Complete list of publications: Publications from PubMed


Sun, X., Yi, Y., Yan, Z., Rosen, B.H., Winter, M.C., Liang, B., Evans, I.A., Rotti, P.G., Yang, Y., Gray, J.S., Park, S.Y., Zhou, W., Zhang, Y., Moll, S.R., Woody, L., Tran, D., Jiang, L., Vonk, A.M., Beekman, J.M., Negulescu, P., Van Goor, F., Fiorino, D., Gibson-Corley, K.N., and Engelhardt, J.F. (2019). In utero and postnatal VX-770 administration rescues multiorgan disease in a ferret model of cystic fibrosis.Science Translational Medicine(Conditionally Accepted, undergoing final editorial revisions)

Yan, Z., Zou, W., Feng, Z., Shen, W., Park, S.Y., Deng, X., Qiu, J., and Engelhardt, J.F.(2019). Establishment of a High Yield rAAV/HBoV Vector Production System Independent of Bocavirus Non-structural Proteins. Hum Gene Ther. (In Press)

Zou, W., Xiong, M., Deng, X., Engelhardt, J.F.,Yan, Z., and Qiu, J. (2019). A Comprehensive RNA-seq Analysis of Human Bocavirus 1 Transcripts in Infected Human Airway Epithelium. Viruses11(1); pii E33.  PMCID: 6357044

Yu, M., Sun, X., Tyler, S.R., Liang, B., Swatek, A.M., Lynch, T.J., He, N., Yuan, F., Feng, Z., Rotti, P.G., Choi, S.H., Shahin, W., Liu, X., Yan, Z., and Engelhardt, J.F.(2019). Highly Efficient Transgenesis in Ferrets Using CRISPR/Cas9-Mediated Homology-Independent Insertion at the ROSA26 Locus Sci Rep9(1): 1971. PMCID:  6374392

Tyler, S.R., Rotti, P.G., Sun, X., Yi, Y., Xie, W., Winter, M.C., Flamme-Wiese, M.J., Tucker, B.A., Mullins, R.F., Norris, A.W., and Engelhardt, J.F.(2019). PyMINEr Finds Gene and Autocrine/Paracrine Networks from Human Islet scRNAseq. Cell Reports26(7): 1951-1964. PMCID: 6394844


Lynch, T.J., Anderson, P.J., Rotti, P.G., Tyler, S.R., Crooke, A.K., Choi, S.H., Montoro, D.T., Silverman, C.L., Shahin, W., Zhao, R., Jensen-Cody, C.W., Adamcakova-Dodd, A., Evans, T.I.A., Xie, W., Zhang, Y., Mou, H., Herring, B.P., Thorne, P.S. and Engelhardt, J.F. (2018) Submucosal gland myoepithelial cells are reserve stem cells that can regenerate mouse tracheal epithelium. Cell Stem Cell, 22(5), pp. 653-667. (An editorial on the manuscript was written in Cell Stem Cell Previews, 22, pp. 613-614). PMCID: 5940330

Rosen, B.H., Evans, T.I.A., Moll, S.R., Gray, J.S., Liang, B., Sun, X., Zhang, Y., Jensen-Cody, C.W., Swatek, A.M. hou, W., He, N., Rotti, P.G., Tyler, S.R., Keiser, N.W., Anderson, P.J., Brooks, L., Li, Y., Pope, R.M., Raiput, M., Hoffman, E.A., Wang, K., Harris, J.K., Parekh, K.R., Gibson-Corley, K.N., and Engelhardt, J.F. (2018). Infection is not required for mucoinflammatory lung disease in CFTR-knockout ferrets. Am J Respir Crit Care Med. 197(10); 1308-1318. (An editorial on the manuscript was written in An J Respir Crit Care Med 2018 Jan 22).PMCID: 5955060

Rotti, P.G., Xie, W., Poudel, A., Yi, Y., Sun, X., Tyler, S.R., Uc, A., Norris, A.W., Hara, M., Engelhardt, J.F., and Gibson-Corley, K.N. (2018). Pancreatic and islet remodeling in cystic fibrosis transmembrane conductance regulator (CFTR) knockout ferrets. Am J Path. 188(4): 876-890. PMCID: 5963477

Bridges, C.S., Miller, P.S., Lidbury, J.A., Suchodolski, J.S., Yi, Y., Engelhardt, J.F., and Steiner, J.M. (2018). Validation of a radioimmunoassay of serum trypsin-like immunoreactivity in ferrets. J. Vet Diagn. Invest. 30(4): 517-522 

O’Malley, Y., Rotti, P.G., Thornell, I.M., Vanegas, Calderon, O.G., Febres-Aldana, C., Durham, K., Yao, J., Li, X., Zhu, Z., Norris, A.W., Zabner, J., Engelhardt, J.F.,and Uc, A. (2018). Development of a polarized pancreatic ductular cell epithelia for physiological studies. J. Appl. Physiol. 125(1): 97-106. PMCID: 6086968

Montoro, D.T., Haber, A.L., Biton, M., Vinarsky, V., Lin, B., Birket, S.E., Yuan F., Chen, S., Leung, H.M., Villoria, J., Rogel, N., Burgin, G., Tsankov, A.M., Waghray, A., Slyper, M., Waldman, J., Nguyen, L., Dionne, D., Rozenblatt-Rosen, O., Tata, P.R., Mou, H., Shivaraju, M., Bihler, H., Mense, M., Tearney, G.J., Rowe, S.M., Engelhardt, J.F.,Regev, A., and Rajagopal, J.  (2018). A revised airway epithelial hierarchy includes CFTR-expressing ionocytes. Nature 560(7718): 319-324. PMCID:  6295155

Johnson, M.B., Sun, X., Kodani, A., Borges-Monroy, R., Girskis, K.M., Ryu, S.C., Wang, P.P., Patel, K., Gonzalez, D.M., Woo, Y.M., Yan, Z., Liang, B., Smith, R.S., Chatterjee, M., Coman D., Papademetris, X., Staib, L.H., Hyder, F., Mandeville, J.B., Grant, P.E., Im, K., Kwak, H., Engelhardt, J.F., Walsh, C.A., and Bae, B.I. (2018). Aspm knockout ferret reveals an evolutionary mechanism governing cerebral cortical size. Nature556(7701): 370-375. PMCID: 6095461

Wang, Z., Cheng, F., Engelhardt, J.F.,Yan, Z., and Qiu, J. (2018). Development of a Novel Recombinant Adeno-Associated Virus Production System Using Human Bocavirus 1 Helper Genes. Mol Ther Methods Clin Dev11:40-51. PMCID: 6205362.


Hackett, T.L., Ferrante,, S.C., Hoptay, C.E., Engelhardt, J.F., Ingram, J.L. Zhang, Y., Alcalca, S.E., Shaheen, F., Matz, E., Pillai, D.S. and Freishtat, R.J. (2017).  A Heterotopic Xenograft model of human airways for investigating fibrosis in asthma. Am J Respir Cell Mol Bio 56(3):291-299. PMCID: 5359536.

Anderson PJ, Lynch TJ, Engelhardt JF. (2017). Multipotent Myoepithelial Progenitor Cells are Born Early During Airway Submucosal Gland Development. Am J Respir Cell Mol Biol. 56(6):716-726. PMCID:  5516291

Wang Z, Shen W, Cheng F, Deng X, Engelhardt JF, Yan Z, Qiu J. (2017). Parvovirus Expresses a Small Noncoding RNA that Plays an Essential Role in Virus Replication. J Virol 91(8). pii: e02375-16. PMCID: 5375684

Guggino, W.B., Benson, J., Seagrave, J., Yan, Z., Engelhardt, J.,Gao, G., Conlon, T.J., Cebotaru, L. (2017) A preclinical study in Rhesus Macaques for Cystic Fibrosis to Assess Gene Transfer and Transduction by AAV1 and AAV5 with a Dual-Luciferase Reporter System. Hum Gene Ther Clin Dev. 28(3): 145-156. PMCID: 5655841

Wang, Z., Deng, X., Zou, W., Engelhardt, J.F.,Yan, Z., and Qiu, J. (2017). Human Bocavirus 1 is a Novel Helper for Adeno-Associated Virus Replication. J Virol. pii: JVI.00710-17. PMCID: 5571279

Yan, Z., Feng, Z., Sun, X., Zhang, Y., Zou, W., Wang, Z., Jensen-Cody, C., Liang, B., Park, S.Y., Qiu, J. and Engelhardt, J.F.(2017). Human Bocavirus Type- Capsid Facilitates the Transduction of Ferret Airways by Adeno-Associated Virus Genomes. Hum Gene Ther 28(8): 612-625. PMCID: 5567599

Sun, X., Yi, Y., Liang, B., Winter, M.C., He, N., Liu, X., Luo, M., Yang, Y., Ode, K.L., Uc., A. Norris, A.W. and Engelhardt, J.F.(2017). CFTR influences beta cell function and insulin secretion through non-cell autonomous exocrine-derived factors. Endocrinology 158(10): 3325-3338. PMCID: 5659686.

Rosen, B.H., Chanson, M., Gawenis, L.R., Liu, J., Sofoluwe, A., Zoso, A. and Engelhardt, J.F.  (2017). Animal and model systems for studying cystic fibrosis. J Cyst Fibros, 17(2S): S28-S34. PMCID: 5858943.

Hao, Z., Zhu, Y., Wang, X., Rotti, P.G., DiMarco, C., Tyler, S.R., Zhao, X, Engelhardt, J.F.,Hone J., Lin, Q. (2017). Real-time monitoring of insulin using a graphene field-effect transistor aptameric nanosensor. ACS Appl Mater Interfaces9(33): 27504-27511

Pillay, S., Zou, W., Cheng, F., Puschnik, A.S., Meyer, N.L., Ganaie, S.S., Deng, X., Wosen, J.E., Davulcu, O., Yan, Z., Engelhardt, J.F.,Brown, K.E., Chapman, M.S., Qiu, J., and Carette, J.E. (2017).  AAV serotypes have distinctive interactions with domains of the cellular receptor AAVR. J Virol. pii: JVI.00391-17. doi: 10.1128/JVI.00391-17. PMCID: 5571256.

Deng, X., Zou, W., Xiong, M., Wang, Z., Engelhardt, J.F., Yem, S.W., Yan, Z., and Qiu, J. (2017). Human parvovirus infection of human airway epithelia induces pyroptotic cell death by inhibiting apoptosis. J Virology, 91(24). PMCID: 5709578

Swatek, A.m., Lynch, T.J., Crooke, A.K., Anderson, P.J., Tyler, S.R., Brooks, L., Ivanovic, M., Klesney-Tait, J.A., Eberlein, M., Pena, T., Meyerholtz, D.K., Engelhardt, J.F., and Parekh, K.R. (2017). Depletion of airway submucosal glands and TP63+KRT5+basal cells in obliterative bronchiolitis. Am J Respir Crit Care Med. 197(8): 1045-1057. PMCID: 5909161


Xu, Q., Choksi, S., Qu, J., Jiang, J., Choe, M., Banfi, B., Engelhardt, J.F., and Liu, Z.G. (2016). NADPH oxidases are essential for macrophage differentiation. JBC 291(38):20030-41. PMCID: 5025689

Yi, Y., Norris, A.W., Wang, K., Sun, X., Uc, A., Moran, A., Engelhardt, J.F., Ode, K.L. (2016). Abnormal glucose tolerance in infants and young children with cystic fibrosis. Am J Respir Crit Care Med. 194(8):974-980. PMCID:  5067820

Deng, X., Xu, P., Zhou, W., Shen, W., Peng, J., Liu, K., Engelhardt, J.F., Yan, ZX., and Qiu, J. (2016). DNA damage signaling is required for replication of the HBoV1 DNA in dividing HEK293 cells. J. Virology16;91(1) pii: e01831-16. PMCID: 5165215

Uc., A., Andersen, D.K., Bellin, M.D., Bruce, J.I., Drewes, A.M., Engelhardt, J.F.,  Forsmark, C.E., Lerch, M.M. Lowe, M.E. Neuschwander-Tetri, B.A., O’Keefe, S.J., Palermo, T.M., Pasricha, P., Saluja, A.K., Singh, V.K., Szigethy, E.M., Whitcomb, D.C., Yadav, D., Cornwell, D.L. (2016). Chronic Pancreatitis in the 21stCentury – Research Challenges and Opportunities: Summary of a National Institute of Diabetes and Digestive and Kidney Diseases Workshop. Pancreas45(10): 1362-1375. PMCID 5117429.

Steines, B., Dickey, D.D., Bergen, J., Excoffon, K.J., Weinstein, J.R., Li, X., Yan, Z., Alaiwa, M.H., Shah, V.S. Bouzek, D.C., Powers, L.S., Gansemer, N.D., Ostedgaard, L.S., Engelhardt, J.F.,Stoltz, D.A., Welsh, M.J., Sinn, P.L., Schaffer, D.V., and Zabner, J. (2016). CFTR gene transfer with AAV improves early cystic fibrosis pig phenotypes. JCI Insight1(14):e88728. PMCID: 5033908.

Sun, Z., Yu, W., Navarro, M.S., Sweat, M., Eliason, S., Sharp, T., Liu, H., Seidel, K., Zhang, L., Moreno, M., Lynch, T., Holton, N.E., Rogers, L., Neff, T., Goodheart, M.J., Michon, F., Klein, O.D., Chai, Y., Dupuy, A., Engelhardt, J.F., Chen, Z., and Amendt, B.A. (2016). Sox2 and Lef-1 interact with Pitx2 to regulate incisor development and stem cell renewal. Development143(22):4115-4126. PMCID 5117215.

Lynch, T.J., Anderson, P.J., Xie, W., Crooke, A.K., Liu, X., Tyler, S.R., Luo, M., Kusner, D.M., Zhang, Y., Neff, T., Burnette, D.C., Walters, K.S., Goodheart, M.J., Parekh, K.R. and Engelhardt, J.F.(2016). Wnt Signaling Regulates Airway Epithelial Stem Cells in Adult Murine Submucosal Glands. Stem Cells34(11): 2758-2771. PMCID:  5809158

Shen, W., Deng, X., Zou, W., Engelhardt, J.F.,Yan, Z. and Qiu, J. (2016). Analysis of the Cis and Trans Requirements for DNA Replication and the right end Hairpin of the human bocavirus 1 genome. J. Virology 90(17):7761-77. PMCID:  4988151

Mou, H. Vinarsky, V., Tata, P.R., Brazauskas, K., Choi, S.H., Crooke, A.K., Zhang, B., Solomon, G. ,M., Turner, B., Bihler, H., Harrington, J., Lapey, A., Channick, C., Keyes, C., Freund, A., Artandi, S., Mense, M., Rowe, S., Engelhardt, J.F.,Hsu, Y.C., and Rajagopal, J. (2016). Duan SMAD signaling inhibition enables long-term expansion of diverse epithelial basal cells. Cell Stem Cell19(2):217-31. PMCID: 4975684

Gibson-Corley, K.N., Meyerholz, D.K., and Engelhardt, J.F. (2016). Pancreatic pathophysiology in cystic fibrosis. J. Pathol. 238(2):311-20. PMCID: 4699289

Evans, T.I., Joo, N.S., Keiser, N.W., Yan, Z., Tyler, S.R., Xie, W., Zhang, Y., Hsiao, J.J., Cho, H.J., Wright, M.E., Wine, J.J. and Engelhardt, J.F. (2016). Glandular Proteome Identifies Antiprotease Cystatin C as a Critical Modulator of Airway Hydration and Clearance. Am J. Respir. Cell Mol. Biol 54(4):469-81. PMCID: 4821051

Deng, X., Yan, Z., Cheng, F., Engelhardt, J.F., and Qiu, J. (2016). Replication of an Autonomous Human Parvovirus in Non-dividing Human Airway Epithelium is facilitated through the DNA Damage and Repair Pathways. PLos PathogI 12(1) e100539. PMCID:  4713420

Zou, W., Cheng, F., Shen, W., Engelhardt, J.F., Yan, Z., and Qiu, J. (2016). Nonstrutural protein NP1 of human bocavirus 1 plays a critical role in the expression of viral capsid proteins. J. Virology 90(9):4658-69. PMCID: 4836317

Yi, Y., Sun, X., Gibson-Corley, K., Xie, W., Liang, B., He, N., Tyler, S.R., Uc, A., Philipson, L.H., Wang, J., Hara, M., Larson Ode, K., Norris, A.W., and Engelhardt J.F. (2016). A transient metabolic recovery from early life glucose intolerance in cystic fibrosis ferrets occurs during pancreatic remodeling. Endocrinology, 157(5): 1852-65. PMCID: 4870869

Spencer, N.Y., Yan, Z., Cong, L., Zhang, Y., Engelhardt, J.F., and Stanton, R.C. (2016). Definitive localization of intracellular proteins: Novel approach using CRISPR-Cas9 genome editing, with glucose 6-phosphate dehydrogenase as a model. Anal Biochem, 1;494:55-67. PMCID: 4695245


Keiser, N.W., Birket, S.E., Evans, I.A., Tyler, S.R., Crooke, A.K., Sun, X., Zhou, W., Nellis, J.R., Stroebele, E.K., Chu, K.K., Tearney, G.J., Stevens, M.J., Harris, J.K., Rowe, S.M., and Engelhardt, J.F. (2015). Defective innate immunity and Hyperinflammation in Newborn CFTR-Knockout Ferret Lungs. Am J Respir Cell Mol Biol. 52(6): 683-694. PMCID: 4491130.

Yan, Z., Stewart, Z.A., Sinn, P.L., Olsen, J.C., Hu, J., McCray, Jr., P.B., and Engelhardt, J.F. (2015). Ferret and pig models of cystic fibrosis: Prospects and promise for gene therapy. Hum Gene Ther Clin Dev, March 2015, 26(1): 38-49. PMCID: 4367511

Uc, A., Olivier, A.K., Griffin, M.A., Meyerholz, D.K., Yao, J., Abu-El-Haija, M., Buchanan, K.M. Vanegas Calderon, O.G., Pezzulo, A.A., Reznikov, K.R., Hoegger, M.J., Rector, M.V., Ostedgaard, L.S., Taft, P.J., Gansemer, N.D., Ludwig, P.S., Hornick, E.E., Stoltz, D.A., Ode, K.L., Welsh, M.J., Engelhardt, J.F. and Norris, A.W. (2015). Glycaemic regulation and insulin secretion are abnormal in cystic fibrosis pigs despite sparing of islet cell mass. Clin Sci (Lond). 128(2): 131-42. PMCID: 4346161

Shen, W., Deng, X., Zou, W., Cheng, F., Engelhardt, J.F., Yan, Z. and Qiu, J. (2015).   Identification and functional analysis of novel nonstructural proteins in human bocavirus 1. J Virology 89(10): 10097-109. PMCID: 4577888

Spencer, N.Y., Yan, Z., Cong, L., Zhang, Y., Engelhardt, J.F., and Stanton, R.C. (2015). Definitive localization of intracellular proteins: Novel approach using CRISPR-Cas9 genome editing, with glucose 6-phosphate dehydrogenase as a model. Analytical Biochemistry 494:55-67.

Wisely, S.M., Ryder, O.A., Santymire, R.M., Engelhardt, J.F., and Novak, B.J. (2015) A Road Map for 21st Century Genetic Restoration: Gene Pool Enrichment of the Black Footed Ferret. J. Hered. 106(5): 581-592. PMCID:  4567841.

Xie, W., Fisher, J.T., Lynch, T.J., Luo, M., Evans, T.I., Neff, T.L., Zhou, W., Zhang, Y., Ou, Y., Bunnett, N.W., Russo, A.F., Goodheart, M.J., Parekh, K.R., Liu, X. and Engelhardt, J.F. (2015)  CGRP induction in cystic fibrosis airways alters the submucosal gland progenitor cell niche in mice. J. Clin. Invest. 125(5): 2179. PMCID:  4463222

Yan, Z., Sun, X., Feng, Z., Li, G., Fisher, J.T., Stewart, Z.A. and Engelhardt, J.F.  (2015). Optimization of recombinant adeno-associated virus-mediated expression for large transgenes, using a synthetic promotor and tandem array enhancers. Human Gene Therapy 26(6): 334-46. PMCID:  4492606

Joo, N.S., Evans, I.A., Cho, H.J., Park, I.H., Engelhardt, J.F., and Wine, J.J. (2015) Proteomic analysis of pure human airway gland mucus reveals a large component of protective proteins. PloS One 10(2):e0116756. PMCID:  4338240

Gibson-Corley, K.N., Meyerholz, D.K., and Engelhardt, J.F. (2015). Pancreative pathophysiology in cystic fibrosis. J. Pathol. (in press)

Evans, T.I., Joo, N.S., Keiser, N.W., Yan, Z., Tyler, S.R., Xie, W., Zhang, Y., Hsiao, J.J., Cho, H.J., Wright, M.E., Wine, J.J. and Engelhardt, J.F. (2015). Glandular Proteome Identifies Antiprotease Cystatin C as a Critical Modulator of Airway Hydration and Clearance. Am J. Respir. Cell Mol. Biol (in press).  


Sun, X., Olivier, A.K., Liang, B., Yi, Y., Sui, H., Evans, T.I.A., Zhang, Y., Zhou, W., Tyler, S.R., Fisher, J.T., Keiser, N.W., Liu, X., Yan, Z., Song, Y., Goeken, J.A., Kinyon, J.M., Fligg, D., Wang, X., Xie, W., Lynch, T.J., Kaminsky, P.M., Stewart, Z.A., Pope, R.M., Frana, T., Meyerholz, D.K., Parekh, K., and Engelhardt, J.F. (2014). Lung Phenotype of Juvenile and Adult CFTR-Knockout Ferrets Am J Respir Cell Mol Biol ;50(3):502-12. PMCID: 4068938

Sun, X., Olivier, A.K., Yi, Y., Pope, C.E., Hayden, H.S., Liang, B., Sui, H., Zhou, W., Hager, K.R., Zhang, Y., Liu, X., Yan, Z., Fisher, J.T., Keiser, N.W., Song, Y., Tyler, S.R., Goeken, J.A., Kinyon, J.M., Radey, M.C., Fligg, D., Wang, X., Xie, W., Lynch, T.J., Kaminsky, P.M., Brittnacher, M.J., Miller, S.I., Parekh, K., Meyerholz, D.K., Hoffman, L.R., Frana, T., Stewart, Z.A., and Engelhardt, J.F. (2014). Gastrointestinal Pathology in Juvenile and Adult CFTR-Knockout Ferrets. American Journal of Pathology 184:1309-1322. PMCID: 4005986.

Spencer, N.Y. and Engelhardt, J.F. (2014). The basic biology of redoxosomes in cytokine-mediated signal transduction and implications for disease-specific therapies. Biochemistry; 53(10):1551-64. PMCID: 3985689.

Sui, H., Yi, Y., Yao, J., Liang, B., Sun, X., Hu, S., Uc, A., Nelson, D.J., Ode, K.L., Philipson, L.H., Engelhardt, J.F., and Norris, A.W. (2014). Quantifying insulin sensitivity and entero-insular responsiveness to hyper- and hypoglycemia in ferrets. PLoS One, 9(3):e90519. PMCID: 3940889

Xie, W., Lynch, T. J., Liu, X., Tyler, S. R., Yu, S., Zhou, X., Luo, M., Kusner, D. M., Sun, X., Yi, Y., Zhang, Y., Goodheart, M. J., Parekh, K. R., Wells, J. M., Xue, H. H., Pevny, L. H., Engelhardt, J. F. (2014). Sox2 modulates Lef-1 expression during airway submucosal gland development. Am J Physiol Lung Cell Mol Physiol, 36(7), L645-60. PMCID: 3962629

Lynch, T.J. and Engelhardt, J.F. (2014). Progenitor Cells in Proximal Airway Epithelial Development and Regeneration. Journal of Cellular Biochemistry 115:1637-1645. PubMed in process. 

Peng, X., Alfoldi, J., Gori, K., Eisfeld, A.J., Tyler, S.R., Tisoncik-Go, J., Brawand, D., Law, L., Skunca, N., Hatta, M., Gasper, D.J., Kelly, S.M. Chang, J., Thomas, M., Johnson, J., Berlin, A.M., Lara, M., Russell, P., Swofford, R., Turner-Maier, J., Young, S., Hourlier T., Aken, B., Searle, S., Sun, S., Yi, Y., Suresh, M., Tumpey, T.M., Siepel, A., Wisely, S.M., Dessimoz, C., Kawaoka, Y., Birren, B.W., Lindblad-Toh, K., Di Palma, F., Engelhardt, J.F., Palermo, R.E. and Katze, M.G. (2014). The draft genome sequence of the ferret (Mustela putorius furo) facilitates study of human respiratory disease. Nature Biotechnology, 32, 1250-1255. PMCID: 4262547


Yan, Z., Sun, X., Evans, I.A., Tyler, S.R., Song, Y., Liu, X., Sui, H., and Engelhardt, J.F. (2013) Postentry processing of recombinant adeno-associated virus type 1 and stransduction of the ferret lung are altered by a factor in airway secretions. Human Gene Therapy 24(3), 786-796. PMCID: 3768237

Fisher, J. T., Tyler, S. R., Zhang, Y., Lee, B. J., Liu, X., Sun, X., Sui, H., Liang, B., Luo, M., Xie, W., Yi, Y., Zhou, W., Song, Y., Keiser, N., Wang, K., de Jonge, H. R., and Engelhardt, J. F. (2013) Bioelectric Characterization of Epithelia from Neonatal CFTR Knockout Ferrets. Am J Respir Cell Mol Biol 49(5), 837-844. PMCID: 3931095

Yan, Z., Lei-Butters, D.C., Keiser, N.W., and Engelhardt, J.F. (2013). Distinct transduction difference between adeno-associated virus type 1 and type 6 vectors in human polarized airway epithelia. Gene Therapy 20(3), 328-37. PMCID: 3443503

Sui, H., Olivier, A.K., Klesney-Tait, J.A., Brooks, L., Tyler, S.R., Sun, X., Skopec, A., Kline, J., Sanchez, P.G., Meyerholz, D.K., Zavazava, N., Iannettoni, M., Engelhardt, J.F. and Parekh, K.R. (2013). Ferret Lung Transplant: An Orthotopic Model of Obliterative Bronchiolitis. Am J. Transplant 13(2), 467-476. PMCID: 3638989

Deng, X., Yan, Z., Luo, Y., Xu, J., Cheng, F. Li, Y., Engelhardt, J.F., and Qiu, J. (2013). In vitro modeling of human bocavirus 1 infection of polarized primary human airway epithelia. Journal of Virology 87(7):4097-102. PMCID: 3624236

Yan, Z., Keiser, N.W., Song, Y., Deng, X., Cheng, F., Qiu, J., and Engelhardt, J.F. (2013). A novel chimeric adeno-associated virus 2 / human bocavirus 1 parvovirus vector efficiently transduces human airway epithelia. Molecular Therapy 20(3), 328-337. PMCID: 3443503

Keiser, N.W. and Engelhardt, J.F. (2013). Gene delivery to the airway. Curr Protoc Hum Genet, Chapter 13. (Unit13 9). PMCID: 3777227.

Spencer, N.Y., Zhou, W., Li, Q., Zhang, Y., Luo, M., Yan, Z., Lynch, T.J., Abbott, D., Banfi, B., and Engelhardt, J.F. (2013). Hepatocytes Produce TNFalpha Following Hypoxia/Reoxygenation and Liver Ischemia/Reperfusion in a NADPH Oxidase- and c-Src-Dependent Manner. Am J Physiol Gastrointest Liver Physiol 305(1), G84-94. PMCID: 3725690


Kaminsky, P.M., Keiser, N.W. Yan, Z., Lei-Butters, D.C.M., and Engelhardt, J.F. (2012). Directing Inegrin-linked Endocytosis of Recombinant AAV enhances Productive FAK-dependent transduction. Molecular Therapy 20(5), 972-983. PMCID: 3345987

Fisher, J.T., Liu, X., Yan, Z., Luo, M., Zhang, Y., Zhou, W., Lee, B.J., Song, Y., Guo, C., Wang, Y., Lukacs, G.L. and Engelhardt, J.F. (2012). Comparative processing and function of human and ferret cystic fibrosis transmembrane conductance regulator. The Journal of Biological Chemistry 287(26), 21673-21685. PMCID: 3381131

Olivier, A.K., Yi, Y., Sun, X., Sui, H., Liang, B., Hu, S., Xie, W., Fisher, J.T., Keiser, N.W., Lei-Butters, D.C., Zhou, W., Yan, Z., Li, G., Evans, T.I.A., Meyerholz, D.K., Wang, K., Stewart, Z.A., Norris, A.W. and Engelhardt, J.F. (2012). Abnormal Endocrine Pancreas Function at Birth in Cystic Fibrosis Ferrets. Journal of Clinical Investigation 122(10):3755–3768. PMCID: 3534166

Huang, Q., Deng, X., Yan, Z., Cheng, F., Luo, Y., Shen, W., Lei-Butters, D.C.M., Chen, A.Y., Li, Y., Tang, L., Soderlund-Venermo, M., Engelhardt, J.F., and Qiu, J. (2012). Establishment of a Reverse Genetics System for the Study of Human Bocavirus and Evidence of Cytopathogenesis in the Polarized Human Airway Epithelium. PLoS Pathogens 8(8), e1002899. PMCID: 3431310

Nakano, Y., Jahan, I., Bonde, G., Sun, X., Hildebrand, M.S., Engelhardt, J.F., Smith, R.J.H., Cornell, R.A., Fritzsch, B., and Bánfi, B. (2012). A Mutation in the Srrm4 Gene Causes Alternative Splicing Defects and Deafness in the Bronx Waltzer Mouse. PloS Genetics, 8(10), e1002966. PMCID: 3464207

Ramsey, B.W., Banks-Schlegel, S., Accurso, F.J., Boucher, R.C., Cutting, G.R., Engelhardt, J.F., Guggino, W.B., Karp, C.L., Knowles, M.R., Kolls, J.K., LiPuma, J.J., Lynch, S., McCray, P.B. Jr., Rubenstein, R.C., Singh, P.K., Sorscher, E. and Welsh, M. (2012). Future Directions in Early Cystic Fibrosis Lung Disease Research. Am J Resp Critic Care Med. 185(8), 887-892. PMCID: 3360572.

Shelton, D. N., Fornalik, H., Neff, T., Park, S. Y., Bender, D., Degeest, K., Liu, X., Xie, W., Meyerholz, D. K., Engelhardt, J. F., Goodheart, M. J. (2012). The Role of LEF1 in Endometrial Gland Formation and Carcinogenesis. PLoS One 7(7), 40312. PMCID: 3391280.

Kaminsky, P.M., Keiser, N.W. Yan, Z., Lei-Butters, D.C.M., and Engelhardt, J.F. (2012). Directing Inegrin-linked Endocytosis of Recombinant AAV enhances Productive FAK-dependent transduction. Molecular Therapy 20(5), 972-983. PMCID: 3345987

Fisher, J.T., Liu, X., Yan, Z., Luo, M., Zhang, Y., Zhou, W., Lee, B.J., Song, Y., Guo, C., Wang, Y., Lukacs, G.L. and Engelhardt, J.F. (2012). Comparative processing and function of human and ferret cystic fibrosis transmembrane conductance regulator. The Journal of Biological Chemistry 287(26), 21673-21685. PMCID: 3381131

Yan, Z., Lei-Butters, D.C., Keiser, N.W., and Engelhardt, J.F. (2012). Distinct transduction difference between adeno-associated virus type 1 and type 6 vectors in human polarized airway epithelia. Gene Therapy. PMCID: 3443503

Olivier, A.K., Yi, Y., Sun, X., Sui, H., Liang, B., Hu, S., Xie, W., Fisher, J.T., Keiser, N.W., Lei-Butters, D.C., Zhou, W., Yan, Z., Li, G., Evans, T.I.A., Meyerholz, D.K., Wang, K., Stewart, Z.A., Norris, A.W. and Engelhardt, J.F. (2012). Abnormal Endocrine Pancreas Function at Birth in Cystic Fibrosis Ferrets. Journal of Clinical Investigation 122(10):3755–3768. PMCID: 3534166

Huang, Q., Deng, X., Yan, Z., Cheng, F., Luo, Y., Shen, W., Lei-Butters, D.C.M., Chen, A.Y., Li, Y., Tang, L., Soderlund-Venermo, M., Engelhardt, J.F., and Qiu, J. Establishment of a Reverse Genetics System for the Study of Human Bocavirus and Evidence of Cytopathogenesis in the Polarized Human Airway Epithelium. PLoS Pathogens 8(8), e1002899. PMCID: 3431310

Nakano, Y., Jahan, I., Bonde, G., Sun, X., Hildebrand, M.S., Engelhardt, J.F., Smith, R.J.H., Cornell, R.A., Fritzsch, B., and Bánfi, B. A Mutation in the Srrm4 Gene Causes Alternative Splicing Defects and Deafness in the Bronx Waltzer Mouse. 2012. PloS Genetics, 8(10). PMCID: 3464207


Fisher, J.T., Zhang, Y., and Engelhardt, J.F. (2011). Comparative biology of cystic fibrosis animal models. Methods Mol Biol 742: 311-334. PMCID: 3617920

Li, Q., Spencer, N.Y., Pantazis, N.J. and Engelhardt, J.F. (2011). ALSIN and SOD1G93A regulate endosomal ROS production by glial cells and pro-inflammatory pathways responsible for neurotoxicity. J. Biol. Chem. 26(24), 40151-40162. PMCID: 3220533

Keiser, N.W., Yan, Z., Zhang, Y., Lei-Butters, D.C.M. and Engelhardt, J.F. (2011). Unique characteristics of AAV1, 2, and 5 Viral Entry, Intracellular Trafficking, and Nuclear Import Define Transduction Efficiency in HeLa Cells. Human Gene Therapy, 22(11), 1433-1444. PMCID: 3225038

Keiser, N.W. and Engelhardt, J.F. (2011). New animal models of cystic fibrosis: what are they teaching us? Curr Opin Pulm Med.17: 478-483. PMCID: 3596000

Li, Y., Wang, J., He, H.Y., Ma, L.J., Zeng, J., Deng, G.C., Liu, X, Engelhardt, J.F., Wang, Y. (2011). Immunohistochemical demonstration of airway epithelial cell markers of Guinea pig. Tissue and Cell 43:283-290. PMCID: 3622455

Xie, W., Fisher, J.T., Lynch, T.J., Luo, M., Evans, T.I.A., Neff, T.L., Zhou, W., Zhang, Y., Ou, Y., Bunnett, N.W., Russo, A.F., Goodheart, M.J., Parekh, K.R., Liu, X., and Engelhardt, J.F. (2011). CGRP Induction in cystic fibrosis airways alters the submucosal gland progenitor cell niche in mice. J Clin Invest. 121(8): 3144-3158. PMCID:3148720

Spencer, NY, Yan, Z, Boudreau, RL, Zhang, Y, Luo, M., Li, Q., Tian, X., Shah, A.M., Davisson, R.L., Davidson, B., Banfi, B. and Engelhardt, J.F. (2011). Control of hepatic nuclear superoxide production by glucose 6-phosphate dehydrogenase and NADPH oxidase-4, The Journal of Biological Chemistry, 286(11), 8977-8987. PMCID:3059029

Zhang, Z. & Liu, X. Wu, T. Liu, J., Zhang, X., Yang, X., Goodheart, M.J., Engelhardt, J.F. and Wang, Y. (2011). Selective suppression of cervical cancer Hela cells by 2-O-β-D-glucopyranosyl-L-ascorbic acid isolated from the fruit of Lycium barbarum L. Cell Biol Toxicol. 27:107–121. PMCID – to be supplied by publisher


Sisson, T.H., Mendez, M., Choi, K., Subbotina, N., Courey, A., Cunningham, A., Dave, A., Engelhardt, J.F., Liu, X., White, E.S., Thannicka, V.J., Moore, B.,B., Christensen, P.J. , and Simon, R.H.. 2009. Targeted Injury of Type II Alveolar Epithelial Cells Induces Pulmonary Fibrosis. Am J Respir Crit Care Med., 181, 254-263. PMCID: 2817814

Sun, X., Sui, H., Fisher, J.T., Yan, Z., Liu, X., Cho, H-J., Joo, N.S., Zhang,Y., Zhou,W., Yi,Y., Kinyon, J.M., Lei-Butters, D.C., Griffin, M.A., Naumann, P., Luo, M., Ascher, J., Wang, K., Frana, T., Wine, J.J., Meyerholz, D.K., and Engelhardt, J.F. (2010). Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis. J. Clinical Invest. 120(9), 3149-3160. PMCID: 2929732

Liu, X., Luo, M., Xie, W., Wells, J.M., Goodheart, M.J., and Engelhardt, J.F. (2010). Sox17 Modulates Wnt3A/b-catenin-Mediated Transcriptional Activation of the Lef-1 Promoter. Am. J. Physiol Lung Cell Mol Physiol. 299:L694-L710. PMCID: 2980392


Oakley, F.D., Smith, R.L., and Engelhardt, J. (2009) Lipid Rafts and Caveolin-1 Coordinate IL-1b-Dependent Activation of NFkB by Controlling Endocytosis of Nox2 and IL-1R1 from the Plasma Membrane. J Biol Chem. 284:48, 33255–33264. PMCID: 2785168

Xu, G., Zhang, Y., Jia, H., Li, J., Liu, X., Engelhardt, J.F., and Wang, Y. 2009. Cloning and identification of microRNAs in bovine alveolar macrophages. Mol Cell Biochem 332:9-16. PubMed - in process

Yan, Z., Lei-Butters, D., Engelhardt, J.F. and Leno, G.H. (2009). Indexing TNF-alpha gene expression using a gene-targeted reporter cell line. BMC Biol, 7:8. PMCID: 2657777

Liu, X., Luo, M., Guo, C., Yan, Z., Wang, Y., Lei-Butters, D.C. and Engelhardt, J.F. (2009). Analysis of Adeno-Associated Virus Progenitor Cell Transduction in Mouse Lung. Molecular Therapy, 17(2), 285-293. PMCID: 2665184 (Supplemental Table 1)

Oakley, F.D., Abbott, D., Li, Q., and Engelhardt, J. (2009). Signaling Components of Redox Active Endosomes: The Redoxosomes. Antioxid Redox Signal. 11(6), 1313-1333. PMCID: 2842130

Yan, Z., Sun, X., and Engelhardt, J.F. (2009). Progress and prospects: techniques for site-directed mutagenesis in animal models. Gene Therapy, 16, 581-588. PMCID: 2798581

Li, Q., Spencer, N.Y., Oakley, F.D., Buettner, G.R., and Engelhardt, J.F. (2009). Endosomal Nox2 Facilitates Redox-Dependent Induction of NFκB by TNFα. Antioxidants and Redox Signaling. 11(6), 1249-1263. PMCID: 2842115

Sun, X., Li, Z., Yi, Y., Ding, W., Chen, J., Engelhardt, J.F., and Leno, G.H. (2009). Chromatin Configurations in the Ferret Germinal Vesicle that Reflect Developmental Competence for In Vitro Maturation. Reprod Domest Anim. 44,320-325. PMCID: 3621713

Carter, B.J., Anklesaria, P., Choi, S., and Engelhardt, J.F. (2009). Redox modifier genes and pathways in amyotrophic lateral sclerosis. Antioxid Redox Signal. 11(7), 1569-1586. PMCID: 2842588


Sun, X., Li, Z., Yi, Y., Chen, J., Leno, G.H., Engelhardt, J.F. (2008). Efficient Term Development of Vitrified Ferret Embryos Using a Novel Pipette Chamber Technique. Biol Reprod. 79(5), 832-840. PMCID: 2658816

Li, Q., Zhang, Y.T., Marden, J.J., Banfi, B., and Engelhardt, J.F. (2008). Endosomal NADPH oxidase regulates c-Src activation following hypoxia/reoxygenation injury. Biochem. J. 411:3, 531-541. PMCID: 3597079

Liu, X., and Engelhardt, J.F. (2008). The glandular stem/progenitor cell niche in airway development and repair. Proc. Am Thorac. Soc., 5(6), 682-688. PMCID: 2645260

Crystal, R.G., Randell, S.H., Engelhardt, J. F., Voynow, J., and Sunday, M.E. (2008). Airway epithelial cells: current concepts and challenge. Proc. Am Thorac. Soc., 5(7), 772-777.

Franks, T.J., Colby, T.V., Travis, W.D., Tuder, R.M., Reynolds, H.Y., Brody, A.R., Cardoso, W.V., Crystal, R.G., Drake, C.J., Engelhardt, J., Frid, M., Herzog, E., Mason, R., Phan, S.H., Randell, S.H., Rose, M.C., Stevens, T., Serge, J., Sunday, M.E., Voynow, J.A., Weinstein, B.M., Whitsett, J. and Williams, M.C. (2008). Resident cellular components of the human lung: current knowledge and goals for research on cell phenotyping and function.Proc. Am Thorac. Soc., 5(7), 763-766

Harraz, M.M., Marden, J.J., Zhou, W., Zhang, Y., Williams, A., Sharov, V.S., Nelson, K., Luo, M., Paulson, H., Schoneich, C., and Engelhardt, J.F. (2008). SOD1 mutations disrupt redox-sensitive Rac regulation of NADPH oxidase in a familial ALS model. J Clin Invest 118: 659-670. PMCID: 2213375

Marden, J.J., Zhang, Y., Oakley, F.D., Zhou, W., Luo, M., Jia, H.P., McCray, P.B., Jr., Yaniv, M., Weitzman, J.B., and Engelhardt J.F. (2008). JunD protects the liver from ischemia/reperfusion injury by dampening AP-1 Transcriptional activation. J Biol Chem. 283(11), 6687-6695. PMCID: 3593645

Sun, X., Yan, Z., Yi, Y., Li, Z., Lei, D., Rogers, C.S., Chen, J., Zhang, Y., Welsh, M., Leno, G.H. Engelhardt, J.F. (2008) AAV Targeted Disruption of the CFTR Gene in Cloned Ferrets. J. Clinic Invest 118:4, 1578-1583. PMCID: 2263147

Rogers, C.S., Hao, Y., Rokhlina, T., Samuel, M., Stoltz, D.A., Li, Y., Petroff, E., Vermeer, D.W., Kabel, A.C., Yan, Z., Spate, L., Wax, D., Murphy, C.N., Rieke, A., Whitworth, K., Linville, M.L., Korte, S.W., Engelhardt, J.F., Welsh, M.J., Prather, R.S. (2008). Production of CFTR-null and CFTR-DeltaF508 heterozygous pigs by adeno-associated virus-mediated gene targeting and somatic cell nuclear transfer, J Clin Invest 118:4, 1571-1577. PMCID: 2265103

Peterson, J.R., Infanger, D.W., Bragga, V.A., Zhang, Y., Sharma, R.V., Engelhardt, J.F., and Davisson, R.L. (2008). Longitudinal non-invasive Monitoring of Transcription Factor Activation in Cardiovascular Regulatory Nuclei Using Bioluminescence Imaging. Physiol Genomics, 33(2), 292-299.

Mumbengegwi, D.R., Li, Q., Li, C., Bear, C.E., and Engelhardt, J.F. (2008). Evidence for a Superoxide Permeability Pathway in Endosomal Membranes. Mol Cell Biol. 28(11): 3700-3712. PMCID: 2423302 .

Rogers, C.S., Abraham, W.M., Brogden, K.A., Engelhardt, J.F., Fisher, J.T., McCray, P.B., Jr., McLennan, G., Meyerholz, D.K., Namati, E., Ostedgaard, L.S., Prather, R.S., Sabater, J.R., Stoltz, D.A.,. Zabner, J., Welsh, M.J. (2008). The porcine lung as a potential model for cystic fibrosis, Am J Physiol Lung Cell Mol Physiol, 295(2), L240-63. PMCID: 2519845

Hsu, M.y., Rovinsky, S.A., Lai, C.Y., Qasem, S., Liu, X., How, J., Engelhardt, J.F., Murphy, G.F. (2008). Aggressive melanoma cells escape from BMP7-mediated autocrine growth inhibition through coordinated Noggin upregulation. Lab Investigation, Aug;88(8):842-55. PMCID: 2676927


Amen, M., Liu, X., Vadlamudi, U., Elizondo, G., Diamond, E., Engelhardt, J.F., and Amendt, B.A. (2007). PITX2 and beta-catenin Interactions Regulate Lef-1 Isoform Expression. Molecular Cell Biology 27(21), 7560-7573. PMCID: 2169058

Marden, J.J., Harraz, M.M., Williams, A.J., Nelson, K., Luo, M., Paulson, H., and Engelhardt, J.F. (2007). Redox Modifier Genes in Amyotrophic Lateral Sclerosis. J Clinical Invest. 17:2913-2919. PMCID: 1974865

Liu, X., Luo, M., Guo, C., Yan, Z., Wang, Y., and Engelhardt, J.F. (2007). Comparative Biology of rAAV Transduction in Ferret, Pig and Human Airway Epithelia. Gene Therapy, 21. 1543-1548. PMCID: 2121584

Liu, X., Luo, M., Trygg, C., Yan, Z., Lei-Butters, D.C.M., Smith, C.B., Fischer, A.C., Munson, K., Guggino, W.B., Bunnell, B.A. and Engelhardt, J.F. (2007). Biological Differences in rAAV Transduction of Human and Old World non-Human Primate Airway Epithelia. Mol Therapy 15(21), 2114-2123. PMCID: 2121582

Harraz, M.M., Park, A., Abbott, D., Zhou, W., Zhang, Y. and Engelhardt, J.F. (2007). MKK6 Phosphorylation Regulates Production of Superoxide by Enhancing Rac GTPase Activity. Antioxidants Redox Signal. 9(11), 1803-1813. PMCID: 3597079

Liu, X., Luo, M., Zhang, L., Ding, W., Yan, Z and Engelhardt, J.F. (2007). Bioelectric Properties of Chloride Channels in Human, Pig, Ferret, and Mouse Airway Epithelia. Am J Resp Cell Mol Biol 36(3), 313-323. (Cover Image). PMCID: 1894945

Driskell, R.R., Goodheart, M., Neff, T., Liu, X., Luo, M., Moothart, C., Sigmund, C.D., Hosokawa, R., Chai, Y., Engelhardt, J.F. (2007). Wnt3a Regulates Lef-1 Expression During Airway Submucosal Gland Morphogenesis. Developmental Biology , 305, 90-102.

Fox, L.E., Green, D., Yan, Z., Engelhardt, J.F ., and Wu, C.F. (2007). Screen for dominant behavioral mutations caused by genomic insertion of p-element transposons in Drosophila: An examination of the integration of viral vector sequences. J Neurogenetics 21(1): 31-43.

Nozoe, M., Hirooka, Y., Koga, Y., Sagara, Y., Kishi, T., Engelhardt, J.F., and Sunagawa, K. (2007). Inhibition of Rac1-Derived Reactive Oxygen Species in Nucleus Tractus Solitarius Decreases Blood Pressure and Heart Rate in Stroke-Prone Spontaneously. Hypertension 50(1), 62-68.

Yan, Z., Lei-Butters, D.C.M., Zhang, Y., Zak, R., and Engelhardt, J.F. (2007). Hybrid Adeno-Associated Virus Bearing Nonhomologous Inverted Terminal Repeats Enhances Dual-Vector Reconstruction of Minigenes In vivo. Human Gene Therapy, 18: 81-87.

Dajani, R., Sanlioglu, S., Zhang, Y., Li, Q., Monick, M.M., Lazartigues, E., Eggleston, T., Davisson, R.L., Hunninghake, G.H. and Engelhardt, J.F. (2007). Pleiotropic Functions of TNF {alpha} Determine Distinct IKK {beta} -Dependent Hepatocellular Fates in Response to LPS. Am J Physiol Gastrointest Liver Physiol.


Liu, X., Driskell, R.R., and Engelhardt, J.F. (2006). Stem Cells in the Lung. Methods Enzymol., 419: 285-321.

Engelhardt, J.F. (2006). AAV Hits the Genomic Bull's-Eye: Adeno-associated Virus Mediates Correction of Genetic Mutations in Mouse Liver. Nature Biotech., 24(8), 949-950.

Yan, Z., Lei-Butters, D.C.M., Liu, X., Zhang, Y., Zhang, L., Luo, M., Zak, R. and Engelhardt, J.F. (2006). Unique Biological Properties of Recombinant AAV1 Transduction in Polarized Human Airway Epithelia. J Biolog Chem., 281(40), 29684-29692.

Hingtgen, S.D. , Yang, J., Dunlay, S.M., Sharma, R.V., Engelhardt, J.F. , and Davisson, R.L. (2006). Nox2-containing NADPH oxidase and AKt activation play a key role in angiotensin II-induced cardiomyocyte hypertrophy. Physiological Genomics, 26, 180-191.

Li Q, Harraz MM, Zhou W, Zhang L, Ding W, Zhang Y, Eggleston T, Yeaman C, Banfi B, and Engelhardt JF. (2006). Nox2 and Rac1 Regulate H2O2-Dependent Recruitment of TRAF6 to Endosomal Interleukin-1 Receptor Complexes. Mol Cell Bio, 26, 140-154 (Cover Image).

Liu X, Yan Z, Luo M, and Engelhardt JF (2006) Species-Specific Differences in Mouse and Human Airway Epithelial Biology of Recombinant Adeno-Associated Virus Transduction. Am J Respir Cell Mol Biol. 34, 56-64 (Cover Image).

Li Q, and Engelhardt, JF. (2006). Interleukin-1beta Induction of NFkappaB is Partially Regulated by H 2 O 2 -mediated Activation of NFkappaB-inducing Kinase. J Bio Chem , 281:3, 1495-1505.

Li Z, Sun X, Chen J, Liu X, Wisely SM, Zhou Q, Renard J-P, Leno GH and Engelhardt JF . (2006). Cloned Ferrets Produced by Somatic Cell Nuclear Transfer. Develop Bio , 293, 439-448.

Ding W, Zhang LN , Yeaman C, and Engelhardt JF (2006). rAAV2 Traffics through both the Late and the Recycling Endosomes in a Dose-Dependent Fashion. Molecular Therapy , 13(4), 671-682.

Li, Z., Sun, X., Chen, J., Leno, G.H. and Engelhardt, J.F. (2006). Factors affecting the efficiency of embryo transfer in the domestic ferret (Mustela putorius furo). Theriogenology, 66(2): 183-90.


Dajani R, Zhang Y, Taft PJ, Travis SM, Starner TD, Olsen A, Zabner J, Welsh, MJ and Engelhardt JF (2005). Lysozyme Secretion by Submucosal Glands Protects the Airway from Bacterial Infection. Am J Respir Cell Mol Biol, 32, 548-552 .

Ding W, Zhang L, Yan Z, and Engelhardt JF . (2005) Intracellular trafficking of adeno-associated viral vectors. Gene Therapy, 12, 873-880.

Liu X, Luo M, Zhang LN, Yan Z, Zak R, Ding W, Mansfield SG, Mitchell LG and Engelhardt JF (2005). Spliceosome-Mediated RNA Trans -Splicing with Recombinant Adeno-Associated Virus Partially Restores Cystic Fibrosis Transmembrane Conductance Regulator Function to Polarized Human Cystic Fibrosis Airway Epithelial Cells. Human Gene Therapy, 16,1116-1123.

Li Z, Sun X, Chen J, Leno GH, and Engelhardt JF. (2005). Factors Affecting the Electrofusion of Mouse and Ferret Oocytes With Ferret Somatic Cells. Mol Rep Dev 72, 40-47.

Li Z, Chen X, Sun X, Zhou Q, Chen J, Leno GH and Engelhardt JF. (2005). Nuclear Transfer of M-phase Ferret Fibroblasts Synchronized with the Microtubule Inhibitor Demecolcine. J Exp Zool 303A:1126-1134.

Lai Y, Yue Y, Liu M, Ghosh A, Engelhardt JF , Chamberlain JS and Duan D. (2005). Efficient in vivo gene expression by trans-splicing adeno-associated viral vectors. Nature Biotech., 23, 1435-1439.

Vadlamudi U, Espinoza HM, Ganga M, Martin DM, Liu X, Engelhardt JF, and Amendt BA. (2005) PITX2, Beta-catenin, and LEF-1 Interact to Synergistically Regulate the LEF-1 Promoter. J. Cell Sci., 118, 1129-1137.

Yan Z., Zak R, Zhang Y, and Engelhardt JF. (2005). Inverted terminal repeat sequences are important for intermolecular recombination and circularization of adeno-associated virus genomes. J Virology 79:1, 364-379.

Panopoulos A. Harraz M, Engelhardt JF and Zandi E (2005). Iron-mediated H2O2 production as a mechanism for cell type-specific inhibition of tumor necrosis factor alpha-induced but not interleukin-1beta-induced IkappaB kinase complex/nuclear factor-kappaB activation. J. Biol. Chem 280: 2912-2923.


Yan Z, Zak R, Zhang Y, Ding W, Godwin S, Munson K, Peluso R, and Engelhardt JF. (2004). Distinct Classes of Proteasome-Modulating Agents Cooperatively Augment rAAV2 and rAAV5-Mediated Transduction from the Apical Surface of Human Airway Epithelia. J Virology, 78:6, 2863-2874.

Fan C, Li Q, Zhang Y, Liu X, Luo M, Abbott D, Zhou W and Engelhardt JF. (2004). I-kappa B-alpha and I-kappa B-beta Possess Injury Context-Specific Functions that Uniquely Influence Hepatic NF-kappaB Induction and inflammation. JCI, 113:5, 746-755 .

Liu X, Yan Z, Luo M, Zak R, Li Z, Driskell RR, Huang Y, Tran N and Engelhardt JF. (2004). Targeted Correction of Single Base-Pair Mutations Using Adeno-Associated Virus Vectors Under Non-Selective Conditions. J Virology, 78:8, 4165-4175.

Sanlioglu AD, Karacay B, Benson PK, Engelhardt JF , and Sanlioglu S. (2004). Novel approaches to augment adeno-associated virus type-2 endocytosis and transduction. Virus Research, 104, 51-59.

Liu X, Driskell RR, Luo M, Abbott D, Filali M, Chen N, Sigmund CD and Engelhardt JF. (2004). Characterization of Lef-1 Promoter Segments that Facilitates Inductive Developmental Expression in Skin. JID, 123:2, 264-274. (Cover Image).

Driskell RR, Liu X, Luo M, Filali M, Abbott D, Chen N, Moothart C, Sigmund CD and Engelhardt JF. (2004). Wnt-Responsive Element Controls Lef-1 Promoter Expression During Submucosal Gland Morphogenesis. Am J Physiol Lung Cell Mol Physiol 287:L752-L763.

Zimmerman MC, Dunlay RP, Lazartigues E, Zhang Y, Sharma RV, Engelhardt JF, Davisson, R.L. (2004). Requirement for Rac1-dependent NADPH oxidase in the cardiovascular and dipsogenic actions of angiotensin II in the brain. Circ. Res., 95, 532-539.

Zhang LN, Karp P, Gerard CJ, Pastor E, Munson K, Yan Z, Godwin S, Thomas C, Zabner , J, Peluso R, Carter B, and Engelhardt JF. (2004). Dual Therapeutic Utility of Proteasome Modulating Agents for Pharmico-Gene Therapy of the Cystic Fibrosis Airway. Molecular Therapy 10 :6, 990-1002.

Terui K, Enosawa S, Haga S, Zhang HQ, Kuroda H, Kouchi K, Matsunaga T, Yoshida H, Engelhardt JF, Irani K, Ohnuma N, and Ozaki M. (2004) Stat3 confers resistance against hypoxia/reoxygenation-induced oxidative injury in hepatocytes through upregulation of Mn-SOD. J Hepatolgy 41 957-965.


Fan C, Li Q, and Engelhardt J.F. 2003. Tyrosine phosphorylation of IkappaBalpha activates NF-kappaB through a redox-regulated and c-Src-dependent. J. Biol. Chem., 278:3, 2072-2080.

Tran , N.D. , Liu, X., Yan, Z., Abbote, D., Jiang, Q., Kmiec, E.B., Sigmund, C.D., and Engelhardt, J.F. 2003. Efficiency of chimeraplast gene targeting by direct nuclear injection using a GFP Recovery Assay. Molecular Therapy 7:2, 248-253.

Duan, D., Yue, Y., and Engelhardt, J.F. 2003. Consequences of DNA-Dependent protein kinase catalytic subunit deficiency on recombinant adeno-associated virus genome circularization and heterodimerization in muscle. J. Virology 77:8, 4751-4759.

Li Z, Sabet MR, Zhou Q, Liu X, Ding W, Zhang Y, Renard J-P, and Engelhardt JF. 2003. Developmental capacity of ferret embryos by nuclear transfer using G0/G1-phase fetal fibroblasts. Biology of Reproduction 68, 2297-2303.

Qiao, L., Han, S.L., Fang, Y., Park, J.S., Gupta, S., Gilfor, D., Amorino, G., Valerie, K., Engelhardt, J.F., Grant, S., Hylemon, P.B. and Dent, P. 2003. Bile Acid Regulation of C/EBP-betta, CREB and c-Jun Function, via the ERK and JNK Pathways, Modulates the Apoptotic Responses of Hepatocytes. Mol Cell Biol 23:9, 3052-3066.

Xiong, S., She, H., Takeuchi, H., Han, B., Engelhardt, J.F., Barton, C.H., Zandi, E., Giulivi, C., and Tsukamoto, H. 2003. Signaling role of intracellular iron in NF-kappaB activation . J. Biol. Chem. 278:17646-17654.

Ding, W., Yan, Z., Zak, R., Saavedra, M., Rodman, D.M. and Engelhardt, J.F. 2003. Second-strand genome conversion of adeno-associated virus type 2 (AAV-2) and AAV-5 is not rate limiting following apical infection of polarized human airway epithelia. J Virology 77:13, 7361-7366.

Li L, Chu Y, Fink GD, Engelhardt JF, Heistad DD, Chen AF. Nov. 2003. Endothelin-1 Stimulates Arterial VCAM-1 Expression via NADPH Oxidase-Derived Superoxide in Mineralocorticoid Hypertension. Hypertension , 997-1003.

Li Z, and Engelhardt JF. Nov. 2003. Progress toward generating a ferret model of cystic fibrosis by somatic cell nuclear transfer. Reprod Biol Endo 1:83. (url: )

Li Z, and Engelhardt JF. Nov. 2003. New technologies for genetic manipulation and animal models: Forum introduction [Review]. Reprod Biol Endo 1:78. (url: )

Yang, J., Marden, J.L., Fan, C., Sanlioglu, S., Weiss, R.M., Ritchie, T.C., Davisson, R.L. and J.F. Engelhardt. 2003. Genetic redox preconditioning differentially modulates AP-1 and NFkB Responses Following Cardiac Ischemia/Reperfusion Injury and Protects Against Necrosis and Apoptosis. Molecular Therapy 7:3, 341-353.


Liu X, Jiang Q, Mansfield SG, Puttaraju M, Zhang Y, Zhou W, Cohn JA, Garcia-Blanco MA, Mitchell LG and Engelhardt JF. Partial correction of endogenous D F508 CFTR in human cystic fibrosis airway epithelial by spliceosome-mediated RNA trans -splicing. Nature Biotechnology 20, 47-52, 2002.

Yan Z, Zak R, Luxton GWG, Ritchie T, Bantel-Schaal U and Engelhardt JF. Ubiquitination of Both Adeno-associated Virus Types 2 and 5 Capsid Proteins Affect the Transduction Efficiency of Recombinant Vectors. Journal of Virology 76(5), 2043-2053, 2002.

Harper S, Hauser MA, Dellorusso C, Duan D, Crawford RW, Phelps SF, Harper HA, Robinson AS, Engelhardt JF, Brooks SV and Chamberlain JS. Modular flexibility of dystrophin: Implications for gene therapy of Duchenne muscular dystrophy. Nature Medicine 8(3), 253-261, 2002.

Yang JQ, Buettner GR, Domann FE, Li Q, Engelhardt JF, Darby Weydert C and Oberley LW. v-Ha-ras Mitogenic Signaling Through Superoxide and Derived Reactive Oxygen Species. Molecular Cardinogenesis 33, 206-218, 2002.

Li Z, Jiang Q, Sabet M, Zhang Y, Ritchie TC and Engelhardt JF. Conditions for In Vitro Maturation and Artificial Activation of Ferret Oocytes. Biology of Reproduction, 66, 1380-1386, 2002.

Yang GS, Schmidt M, Yan Z, Lindbloom JD, Harding TC, Donahue BA, Engelhardt JF, Kotin R and Davidson BL. Virus-Mediated Transduction of Murine Retina with Adeno-Associated Virus: Effects of Viral Capsid and Genome Size. J. Virology 76: 15, 7651-7660, 2002.

Filali, M., Cheng, N., Abbott, D., Leontiev, V., and Engelhardt, JF. Wnt-3A/beta-catenin signaling induces transcription from the LEF-1 promoter. J. Biol. Chem. 277:36, 33398-33410, 2002.

Engelhardt JF. The lung as a metabolic factory for gene therapy. J. Clin. Inv. 110, 429-432, 2002.

Doerschug K, Sanlioglu S, Flaherty DM, Wilson RL, Yarovinsky T, Monick MM, Engelhardt JF, and Hunninghake GW. First-Generation Adenovirus Vectors Shorten Survival Time in a Murine Model of Sepsis. J. Immunol. 169:6539-6545, 2002.

Fan, C., Yang, J., and Engelhardt, J.F. 2002. Temporal Pattern of NFkappaB Activation Influences Apoptotic Cell Fate in a Stimuli Dependent Fashion. Journal of Cell Science 115, 4843-4853.


Ritchie T, Zhou W, McKinstry E, Hosch M, Zhang Y, Nathke I, and Engelhardt JF. Developmental expression of catenins and associated proteins during submucosal gland morphogenesis in the airway. Experimental Lung Research 27 :121-141, 2001.

Wang X, Zhang Y, Amberson A, and Engelhardt JF. New models of the tracheal airway define the glandular contribution to airway surface fluid and electrolyte composition. Am. J. Resp. Cell. Mol. Biol. 24:195-202, 2001.

Zanetti M, Zwacka RM, Engelhardt JF, Katusic Z, and O'Brien T. Superoxide anions and endothelial cell proliferation in normoglycemia and hyperglycemia. Anterioscler Thromb Vasc Biol 21:195-200, 2001.

Sanlioglu S, Monick MM, Luleci G, Hunninghake GW and Engelhardt, JF. Rate limiting steps of AAV transduction and implications for human gene therapy. Current Gene Therapy 1:137-147, 2001.

Engelhardt JF. Stem cells niches in the mouse airway. Am. J. Respir. Cell. Mol. Biol 24:649-652, 2001.

Duan D, Yan Z, Yue Y, Ding W, and Engelhardt JF. Enhancement of Muscle Gene Delivery with Pseudotyped Adeno-Associated Virus Type 5 Correlates with Myoblast Differentiation. Journal of Virology 75(16):7662-7671, 2001.

Li Q, Sanlioglu S, Li S, Ritchie T, Oberley L, and Engelhardt JF. GPx-1 gene delivery modulates NFk B activation following diverse environmental injuries through a specific subunit of the IKK complex. Antioxidant and Redox Signaling 3(3):415-432, 2001.

Engelhardt JF, Sen CK, and Oberley L. Redox-modulating gene therapies for human diseases Antioxidant and Redox Signaling 3(3):341-346, 2001.

Zhou W, Zhang Y, Hosch MS, Lang A, Zwacka RM, and Engelhardt JF. Subcellular site of superoxide dismutase expression differentially controls AP-1 activity in the liver and injury following ischemia/reperfusion. Hepatology 33(4):902-914, 2001.

Sanlioglu S, Williams C, Samavati L, Butler N, Wang G, McCray P Jr., Ritchie T. Hunninghake G, Zandi E, and Engelhardt JF. Lipopolysaccharide Induces Rac1-dependent Reactive Oxygen Species Formation and Coordinates Tumor Necrosis Factor- a Secretion through IKK Regulation of NF- k B. J. Biol. Chem. 276(32): 30188-30198, 2001.

Duan D, Yue Y, and Engelhardt JF. Expanding AAV Packaging Capacity with Trans-splicing or Overlapping Vectors: A Quantitative Comparison. Molecular Therapy 4(4):383-391, 2001.

Li ZY, Jiang QS, Zhang YL, Liu XM, and Engelhardt JF. Successful Production of Offspring Following Superovulation and in vitro Culture of Embryos from Domestic Ferrets. Reproduction 122:611-618, 2001.

Khubchandani, KR, Goss, KL, Engelhardt JF and Snyder, JM. In situ hybridization of SP-A mRNA in adult human conducting airways. Pediatr Pathol Mol Med 20(5): 349-66, 2001.


Walters RW, Duan D, Engelhardt JF, and Welsh MJ. Incorporation of Adeno-Associated Virus in a Calcium Phosphate Coprecipitate Improves Gene Transfer to Airway Epithelia In Vitro and In Vivo. J. Virology , 74:535-540, 2000.

Naren AP, Anke D, Cormet-Boyaka E, Boyaka PN, McGhee JR, Zhou W, Akagawa K, Jujiwara T, Thome U, Engelhardt JF, Nelson DJ, and Kirk KL. Syntaxin 1A is Expressed in Airway Epithelial Cells Where it Modulates CFTR Cl - Currents. J. Clin. Inv . 105:377-386, 2000.

Sanlioglu S, Benson P, and Engelhardt JF. Loss of ATM Function Enhances Recombinant Adeno-Associated Virus Transduction and Integration through Pathways Similar to UV Irradiation . Virology , 268:68-78, 2000. (Cover Image)

Duan D, Yue Y, Yan Z, and Engelhardt JF. A New Dual Vector Approach to Enhance Recombinant AAV Mediated Gene Expression Through Intermolecular Cis-activation. Nature Med. 6:595-598, 2000.

Yan Z, Zhang Y, Duan D, and Engelhardt JF. Trans-Splicing Vectors Expand the Utility of Adeno-Associated Virus for Gene Therapy. PNAS 97:6716-6721, 2000.

Jiang Q, Li J, Dubroff R, Ahn YJ, Foskett, JK, Engelhardt JF, Kleyman, TR. Epithelial sodium channels regulate cystic fibrosis transmembrane conductance regulator chloride channels in Xenopus oocytes . J. Biol. Chem. 275:13266-13274, 2000.

Duan D, Yue Y, Yan Z, Yang J, and Engelhardt JF. Endosomal Processing Limits Gene Transfer to Polarized Airway Epithelia by Adeno-Associated Virus. J. Clin. Inv . 105:1573-1587, 2000. (Cover Image)

Lam EWN, Hammad HM, Zwacka R, Darby CJ, Baumgardner KR, Davidson BL, Oberley TD, Engelhardt JF, and Oberley LW. Immunolocalization and adenoviral vector-mediated manganese superoxide dismutase gene transfer to experimental oral tumors. J. Dent. Res. 79:1410-1417, 2000.

Sanlioglu, S., P.K. Benson, J. Yang, E.M. Atkinson, T. Reynolds, and J.F. Engelhardt. Endocytosis and nuclear trafficking of adeno-associated virus type 2 are controlled by rac1 and phosphatidylinositol-3 kinase activation. J Virol. 74:9184-9196. 2000.


Epperly, MW, Bray, JA, Krager, S, Berry , LM, Gooding, W, Engelhardt, JF, Zwacka, R, Travis, EL, and Greenberger, JS. Intratracheal injection of adenovirus containing the human MnSOD transgene protects athymic nude mice from irradiation-induced organizing alveolitis. Int. J. Radiation Oncology Biol. Phys . 43:169-181 (1999).

Duan D, Sharma P, Dudus L, Zhang Y, Sanlioglu S, Yan Z, Yue Y, Ye Y, Lester R, Yang J, Fisher KJ and Engelhardt JF. Formation of Adeno-Associated Virus Circular Genomes is Differentially Regulated by Adenovirus E4-ORF6 and E2a Gene Expression. J. Virology 73:161-169 (1999).

Zhang Y and Engelhardt JF. Airway Surface Fluid Volume and Chloride Content in Cystic Fibrosis and Normal Bronchial Xenografts. Am. J. Physiol: Cell Physiol 276: C469-C476 (1999).

Sanlioglu S, Duan D, and Engelhardt JF. Two independent molecular pathways for rAAV genome conversion occur following UV-C and E4orf6 augmentation of rAAV transduction. Human Gene Therapy 10:591-602 (1999).

Duan D, Yue Y, and Engelhardt JF: Response to "Polarity Influences the efficiency of recombinant adeno-associated virus infection in differentiated airway epithelia". Human Gene Therapy 10:1553-1557 (1999).

Sanlioglu, S, and Engelhardt JF. Cellular Redox State Alters Recombinant Adeno-Associated Virus Transduction Through Tyrosine Phosphatase Pathways. Gene Therapy 6:1427-1437, 1999.

Duan, D, Yan, Z, Yue, Y, and Engelhardt, JF. Structural analysis of AAV transduction circular intermediates. Virology 261:8-14, 1999.

Lam EW, Zwacka R, Seftor EA, Nieva DRC, Davidson BL, Engelhardt JF, Hendrix MJC, and Oberley LW. Effects of antioxidant enzyme overexpression on the invasive phenotype of hamster cheek pouch carcinoma cells. Free Radical Biol. & Med . 27:572-579, 1999.

Yang J, Zhou W, Zhang Y, Zidon T, Ritchie T, and Engelhardt JF. Concatamerization of Adeno-Associated Virus Circular Genomes Occurs through Intermolecular Recombination. J Virology . 73:9468-9477, 1999.

Duan D, Yue Y, Zhou W, Labed B, Ritchie T, Grosschedl R, and Engelhardt JF: Submucosal Gland Development in the Airway is Controlled by Lymphoid Enhancer Binding Factor-1 (Lef-1). Development, 126:4441-4453, 1999.

Duan D, Li Q, Kao A, Yue Y, Pessin J, and Engelhardt JF.Dynamin is Required for Recombinant Adeno-associated Virus Type 2 Infection. J Virology , 73:10371-10376, 1999.


Sharma P, Dudus L, Nielsen PA, Clausen H, Yankaskas JR, Hollingworth MA, and Engelhardt JF. MUC5B and MUC7 are Differentially Expressed in Mucous and Serous Cells of Submucosal Glands in Human Bronchial Airways. Am. J. Resp. Cell. Mol. Biol. 19:30-37 (1998). (Cover Image)

Duan D, Sehgal A, Yao J, and Engelhardt JF. The Lef1 transcription factor expression defines airway progenitor cell targets for in utero gene therapy of submucosal gland in cystic fibrosis. Am. J. Resp. Cell. Mol. Biol. 18:750-758 (1998).

Zwacka RM, Dudus L, Epperly MW, Greenberger JS, and Engelhardt JF. Redox Gene Therapy Protects Human IB-3 Lung Epithelial Cells Against Ionizing Radiation-Induced Apoptosis. Human Gene Therapy 9:1381-1386 (1998).

Zhang Y, Jiang Q, Dudus, L, Yankaskas JR, and Engelhardt JF. Vector Specific Complementation Profiles of Two Independent Primary Defects in Cystic Fibrosis Airways. Human Gene Therapy 9:635-648 (1998).

Gonzalez-Zulueta, M, Mukhin G, Zwacka RM, Engelhardt JF, Oberley LW, Dawson VL, and Dawson TM. Manganese Superoxide dismutase protects nNOS Neurons from NMDA and Nitric Oxide Mediated Neurotoxicity. J. Neuroscience 18:2040-2055 (1998).

Epperly M, Bray J, Kraeger S, Zwacka R, Engelhardt JF, Travis E, and Greenberger J: Prevention of Late Effects of Irradiation Lung Damage by Manganese Superoxide Dismutase Gene Therapy. Gene Therapy 5:196-208 (1998).

Fang X, Weintraub NL, Rios CD, Chappell DA, Zwacka RM, Engelhardt JF, Oberley LW, Yan T, Heistad DD, and Spector AA. Overexpression of Human Superoxide Dismutase Inhibits Oxidation of Low-Density Lipoprotein by Endothelial Cells. Circulation Research 82:1289-1297 (1998).

Zwacka RM, Zhou W, Zhang Z, Darby CJ, Dudus L, Halldorson J, Oberley L, and Engelhardt JF. Redox Gene Therapy of Liver Ischemia/Reperfusion Injury Reduces AP1 and NFkB Activation. Nature Medicine 4:698-704 (1998).

Duan D, Sharma P, Yang J, Y Yue, Dudus L, Zhang Y, Fisher KJ, and Engelhardt JF. Circular Intermediates of Recombinant Adeno-Associated Virus have Defined Structural Characteristics Responsible for Long Term Episomal Persistence In Muscle Tissue. J. Virology 72:8568-8577 (1998).

Zwacka RM, Zhang Y, Halldorson J, and Engelhardt JF. Ischemia/reperfusion injury in the liver of BALB/c mice activates AP-1 and NF- k B Independently of I k B Degradation. Hepatology 28:1022-1030 (1998).

Jiang Q, Mak D, Devidas S, Schwiebert EM, Bragin A, Zhang Y, Skach WR, Guggino WB, Foskett JK, and Engelhardt JF. Cystic fibrosis transmembrane conductance regulator-associated ATP release is controlled by a chloride sensor. J.Cell Biology 143(3):645-57 (1998).

Ceneviva GD, Tzeng E, Hoyt DG, Yee E, Gallagher A, Engelhardt JF, Kim YM, Billiar TR, Watkins SA, and Pitt BR. Nitric oxide inhibits lipopolysaccharide-induced apoptosis in pulmonary artery endothelial cells. Am. J. Physiol 275:L717-28 (1998).

Duan D, Yue Y, McCray PB, and Engelhardt JF. Polarity Influences the efficiency of recombinant adeno-associated virus infection in differentiated airway epithelia. Human Gene Therapy 9:2761-2776 (1998).

Woo YJ, Zhang JC, Vijayasarathy C, Zwacka RM, Engelhardt JF, Gardner TJ, Sweeney HL. Recombinant adenovirus-mediated cardiac gene transfer of superoxide dismutase and catalas attenuates postischemic contractile dysfunction. Circulation 19:II255-60 (1998).


Presente A, Sehgal A, Dudus L, and Engelhardt J. Differentially Regulated Epithelial Expression of an Eph Family Tyrosine Kinase (Fek2) During Tracheal Surface Airway and Submucosal Gland Development. Am. J. Resp. Cell. and Mol. Biol. 16;53-61 (1997).

Duan D, Fisher K, Burda J, and Engelhardt JF. Structural and Functional Heterogeneity of Integrated Recombinant AAV Genomes. Virus Research 48;41-56 (1997).

Zwacka R, Zhang Y, Halldorson JB, Schlossberg H, Dudus D, and Engelhardt JF. CD4 + T-Lymphocytes Mediate Ischemia/Reperfusion-Induced Inflammatory Responses in Mouse Liver. J. Clin. Inv. 100;279-289 (1997).

Bennett J, Duan D, Engelhardt J, and Maguire AM. Real-time Non-Invasive in vivo Assessment of Adeno-Associated Virus Mediated Retinal Transduction. Invest. Opthom. and Visual Sci. 38:2857-2863 (1997).

Lam E, Zwacka R, Engelhardt JF, Davidson B, Domann F, and Oberley LW. Adenoviral-Mediated Manganese superoxide dismutase gene transfer to hamster cheek pouch carcinoma cells. Cancer Research 57:5550-5556 (1997).


Zhang Y, Yankaskas J, Wilson JM, and Engelhardt JF.In vivo Analysis of Fluid Transport in Cystic Fibrosis Airway Epithelia of Bronchial Xenografts. Am. J. Physiology: Cell Physiology 270:C1326-C1335 (1996).

Schlossberg H, Zhang Y, Dudus L, and Engelhardt JF. Expression of c-fos and c-jun During Hepatocellular Remodeling Following Ischemia Reperfusion in Mouse Liver. Hepatology 23;1546-1555 (1996).

Sehgal A, Presente A, Dudus L, and Engelhardt JF. Isolation of differentially expressed cDNAs during ferret tracheal development: Application of Differential Display PCR. Experimental Lung Research 22;419-434 (1996).

Sehgal A, Presente A, and Engelhardt JF. Developmental Expression Patterns of CFTR in Ferret Tracheal Surface Airway and Submucosal Glands Am. J. Resp. Cell. and Mol. Biol. 15;122-131 (1996).

Presente A, Sehgal A, Dudus L, and Engelhardt J: Differentially Regulated Epithelial Expression of an Eph Family Tyrosine Kinase (Fek2) During Tracheal Surface Airway and Submucosal Gland Development Am. J. Resp. Cell. and Mol. Biol. 16;53-61 (1997).


Pilewski, JM., Engelhardt JF., Bavaria JE., Kaiser LR., Wilson JM., and Albelda S.M. Adenovirus-mediated gene transfer to human bronchial submucosal glands using xenografts. Amer. J. Physiol: Lung Cell and Mol Physiol. 12:L657-L665, (1995)

Engelhardt JF, Schlossberg H, Yankaskas JR, and Dudus L. Progenitor cells of the adult human airway involved in submucosal gland development. Development 121:2031-2046 (1995).

Goldman MJ., Litzky LA., Engelhardt JF., and Wilson JM. Transfer of the CFTR Gene to the Lung of Nonhuman Primates with E1-Deleted, E2a-Defective Recombinant Adenovirus: A Preclinical Toxicology Study. Human Gene Therapy 6:839-851 (1995).

Knowles MR., Hohneker KW., Zhou RN., Olsen J., Hoah TL., Hu PC., Leigh MW., Engelhardt JF., Edwards LJ., Jones KR., Grossman M., Wilson JM, Johnson LG., and Boucher RC. A controlled study of adenoviral-vector-mediated gene transfer in the nasal epithelium of patients with cystic fibrosis. New England Journal of Medicine 333:823-31 (1995).

Zhang Y, Doranz B, Yankaskas JR, and Engelhardt JF. Genotypic analysis of respiratory mucous sulfation defects in cystic fibrosis. J. Clin. Inv. 96:2997-3004 (1995).


Grossman M, Raper SE, Kozarsky K, Stein EA, Engelhardt JF, Muller D, Lupien PJ, and Wilson JM. Successful ex vivo gene therapy directed to liver in a patient with familial hypercholesterolaemia, Nature Genetics. 6:335-341, (1994).

Wilson JM, Engelhardt JF, Grossman M, Simon RH, and Yang Y. Gene Therapy of Cystic Fibrosis Lung Disease Using E1 Deleted Adenoviruses: A Phase I Trial. Human Gene Therapy, 5:501-519 (1994).

Boucher RC, Knowles MR, Johnson LG, Olsen JC, Pickles R, Wilson JM, Engelhardt JF, Yang Y, and Grossman M. Gene Therapy for Cystic Fibrosis Using E1-Deleted Adenovirus: A Phase I Trial in the Nasal Cavity. Human Gene Therapy , 5:615-639 (1994).

Engelhardt JF, Ye X, Doranz B, and Wilson JM. Ablation of E2A in recombinant adenoviruses improves transgene persistence and decreases inflammatory response in mouse liver, PNAS , 91;6196-6200 (1994).

Yang Y, Engelhardt JF, and Wilson JM. Ultrastructural localization of variant forms of CFTR in human bronchial epithelia of xenografts, Am. J. Resp. Cell and Mol. Biol ., 11;7-15 (1994).

Yang Y, Nunes FA, Berencsi K, Gonczol E, Engelhardt JF and Wilson JM. Inactivation of E2a in Recombinant Adenoviruses Improves the Prospect or Gene Therapy of Cystic Fibrosis. Nature Genetics, 7;362-369 (1994).

Engelhardt JF, Smith SS, Allen E, Yankaskas JR, Dawson DC and Wilson JM. Coupled secretion of chloride and mucus in skin of Xenopus laevis: possible role for CFTR Amer J Physiol: Cell, 267;C491-C500, (1994).

Engelhardt JF, Litsky L, and Wilson JM. Prolonged Transgene Expression in Cotton Rat Lung with Recombinant Adenoviruses Defective in E2a. Human Gene Therapy, 5;1217-1229 (1994).

Grubb BR, Pickles RJ, Ye H, Yankaskas JR, Vick RN, Engelhardt JF, Wilson JM, Johnson, LG,and Boucher, RC.Inefficient gene transfer by adenovirus vector to cystic fibrosis airway epithelia of mice and humans. Nature 371;802-806 (1994)

Root KV, Engelhardt JF, Post M, Wilson JM, and Van Dyke RW. CFTR does not alter acidification of L cell endosomes. Biochem Biophys. Res Comm. 205;396-401, (1994).

Engelhardt JF, Zepeda M, Cohn JA, Yankaskas JR and Wilson JM. Expression of the cystic fibrosis gene in adult human lung. J. Clin. Inv., 93:737-749, (1994).


Yang Y, Raper SE, Cohn JA, Engelhardt JF and Wilson JM. An approach for treating the hepatobillary disease of cystic fibrosis by somatic gene transfer. Proc Natl Acad Sci USA 90:4601-4605, (1993).

Yang Y, Devor DC, Engelhardt JF, Ernst SA, Strong TV, Collins F, Cohn JA, Frizzell RA and Wilson JM. Molecular basis of defective anion transport in L cells expressing recombinant forms of CFTR. Human Molecular Genetics 2:1253-1261, (1993)

Engelhardt JF, Simon RH, Yang Y, Zepeda M, Pendleton SW, Doranz B, Grossman M and Wilson JM. Adenovirus-Mediated Transfer of the CFTR Gene to Lung of Non-human Primates: Biological Efficacy Study. Human Gene Therapy , 4:759-769, (1993).

Simon RH, Engelhardt JF, Yang Y, Zepeda M, Pendleton SW, Grossman M and Wilson JM. Adenovirus-Mediated Transfer of the CFTR Gene to Lung of Non-human Primates: Toxicity Study. Human Gene Therapy , 4:771-780, (1993).

Engelhardt JF, Yang Y, Stratford-Perricaudet LD, Allen ED, Kosarsky K, Perridaudet M, Yankaskas JR and Wilson JM: Direct gene transfer of human CFTR into human bronchial epithelia of xenografts with E1 deleted adenoviruses. Nature Genetics 4:27-33, (1993).


Whitsett JA, Dey C, Wikenheiser KA, Clark JC, Wert ES, Gregory RJ, Smith AE, Cohn JA,Wilson JM, and Engelhardt JF. Human cystic fibrosis transmembrane conductance regulator directed to respiratory epithelial cells of transgenic mice. Nature Genetics 2:13-20, (1992).

Engelhardt JF, Yankaskas JR, Ernst SA, Yang Y, Marino CR, Boucher RC, Cohn JA and Wilson JM. Submucosal glands are the predominant site of CFTR expression in the human bronchus. Nature Genetics 2:240-248, (1992).

Engelhardt JF, Yankaskas JR, and Wilson JM. In vivo retroviral gene transfer into human bronchial epithelia of xenografts. J Clin. Inv. 90:2598-2607 (1992).

Engelhardt JF, Yang Y, Stratford-Perricaudet LD, Allen ED, Kosarsky K, Perridaudet M, Yankaskas JR and Wilson JM. Direct gene transfer of human CFTR into human bronchial epithelia of xenografts with E1 deleted adenoviruses. Nature Genetics 4:27-33, (1993).


Engelhardt JF, Steel G and Valle D. Transcriptional analysis of ornithine aminotransferase gene promoter. J. Biol. Chem. 266:752-758, (1991).

Engelhardt JF, Allen E and Wilson JM. Reconstitution of tracheal grafts with a genetically modified epithelium. PNAS 88:11192-11196, (1991).


Mitchell GA, Brody LC, Engelhardt JF, Steel G, Obie C, Fontaine G, Saudubray J-M, Bonnefont J-P, Labuda D and Valle D. Inactivation of ornithine- d -aminotransferase by splice mediated insertion of Alu sequences: A new role for Alu in humans mutations? Ped Res 24, (1990).

Engelhardt JF, Bisat F, Kellum MJ, and Pitha PM.Retrovirus Vector-Targeted inducible expression of Human b -Interferon Gene to B-Cells. Virology 178:419-428, (1990).


Mitchell GA, Brody LC, Sipila I, Looney JE, Wong C, Engelhardt JF, Patel AS , Steel G, Obie C, Kaiser-Kupfer MI and Valle D. At least two mutant alleles of ornithine- d -aminotransferase cause gyrate atrophy of the choroid and retina in Finns. Proc Natl Acad Sci USA 86:197-201, (1989).

Raj BN, Engelhardt JF, Au W-C, Levy ED and Pitha PM. Virus infection and interferon can activate gene expression through a single synthetic element but endogenous genes show distinct regulation. J. Biol. Chem. 264:16658-16666, (1989).


Mitchell GA, Looney JE, Brody LC, Steel G, Suchanek M, Engelhardt JF, Willard HF and Valle D. Human ornithine- d -aminotransferase: cDNA cloning and analysis of the structural gene. J Biol Chem 263:14288-14295, (1988).