Progress toward understanding the pathogenesis of cystic fibrosis (CF) and developing effective therapies has been hampered by lack of a relevant animal model. CF mice fail to develop the lung and pancreatic disease that cause most of the morbidity and mortality in patients with CF. Pigs may be better animals than mice in which to model human genetic diseases because their anatomy, biochemistry, physiology, size, and genetics are more similar to those of humans. However, to date, gene-targeted mammalian models of human genetic disease have not been reported for any species other than mice. Here we describe the first steps toward the generation of a pig model of CF. We used recombinant adeno-associated virus (rAAV) vectors to deliver genetic constructs targeting the CF transmembrane conductance receptor (CFTR) gene to pig fetal fibroblasts. We generated cells with the CFTR gene either disrupted or containing the most common CF-associated mutation (ΔF508). These cells were used as nuclear donors for somatic cell nuclear transfer to porcine oocytes. We thereby generated heterozygote male piglets with each mutation. These pigs should be of value in producing new models of CF. In addition, because gene-modified mice often fail to replicate human diseases, this approach could be used to generate models of other human genetic diseases in species other than mice.
Christopher S. Rogers, Yanhong Hao, Tatiana Rokhlina, Melissa Samuel, David A. Stoltz, Yuhong Li, Elena Petroff, Daniel W. Vermeer, Amanda C. Kabel, Ziying Yan, Lee Spate, David Wax, Clifton N. Murphy, August Rieke, Kristin Whitworth, Michael L. Linville, Scott W. Korte, John F. Engelhardt, Michael J. Welsh, Randall S. Prather
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BMC Biotechnology | 2013 |
Air Trapping and Airflow Obstruction in Newborn Cystic Fibrosis Piglets
RJ Adam, AS Michalski, C Bauer, MH Alaiwa, TJ Gross, MS Awadalla, DC Bouzek, ND Gansemer, PJ Taft, MJ Hoegger, A Diwakar, M Ochs, JM Reinhardt, EA Hoffman, RR Beichel, DK Meyerholz, DA Stoltz |
American journal of respiratory and critical care medicine | 2013 |
Genetically modified pigs to model human diseases
T Flisikowska, A Kind, A Schnieke |
Journal of Applied Genetics | 2013 |
Natural genotypes via genetic engineering
KD Wells |
Proceedings of the National Academy of Sciences | 2013 |
Early Airway Structural Changes in Cystic Fibrosis Pigs as a Determinant of Particle Distribution and Deposition
M Awadalla, S Miyawaki, MH Alaiwa, RJ Adam, DC Bouzek, AS Michalski, MK Fuld, KJ Reynolds, EA Hoffman, CL Lin, DA Stoltz |
Annals of Biomedical Engineering | 2013 |
Immunization of aged pigs with attenuated pseudorabies virus vaccine combined with CpG oligodeoxynucleotide restores defective Th1 immune responses
F Ming, J Yang, P Chu, M Ma, J Shi, H Cai, C Huang, H Li, Z Jiang, H Wang, W Wang, S Zhang, L Zhang |
PloS one | 2013 |
Production of cloned pigs with targeted attenuation of gene expression
V Bordignon, N El-Beirouthi, BG Gasperin, MS Albornoz, MA Martinez-Diaz, C Schneider, D Laurin, D Zadworny, LB Agellon |
PloS one | 2013 |
CFTR-deficient pigs display peripheral nervous system defects at birth
LR Reznikov, Q Dong, JH Chen, TO Moninger, JM Park, Y Zhang, J Du, MS Hildebrand, RJ Smith, CO Randak, DA Stoltz, MJ Welsh |
Proceedings of the National Academy of Sciences | 2013 |
The Cystic Fibrosis Gene: A Molecular Genetic Perspective
LC Tsui, R Dorfman |
Cold Spring Harbor Perspectives in Medicine | 2013 |
From in vitro to in vivo Models of Bacterial Biofilm-Related Infections
D Lebeaux, A Chauhan, O Rendueles, C Beloin |
Pathogens | 2013 |
Sinus hypoplasia precedes sinus infection in a porcine model of cystic fibrosis
EH Chang, AA Pezzulo, DK Meyerholz, AE Potash, TJ Wallen, LR Reznikov, JC Sieren, PH Karp, S Ernst, TO Moninger, ND Gansemer, PB McCray, DA Stoltz, MJ Welsh, J Zabner |
The Laryngoscope | 2012 |
Cystic fibrosis: a mucosal immunodeficiency syndrome
TS Cohen, A Prince |
Nature Medicine | 2012 |
Advances in Cell and Gene-based Therapies for Cystic Fibrosis Lung Disease
M Oakland, PL Sinn, PB McCray |
Molecular Therapy | 2012 |
Why mouse airway submucosal gland serous cells do not secrete fluid in response to cAMP stimulation
RJ Lee, JK Foskett |
The Journal of biological chemistry | 2012 |
Generation of SV40-transformed rabbit tracheal-epithelial-cell-derived blastocyst by somatic cell nuclear transfer
D Semir, R Maurisse, F Du, J Xu, X Yang, B Illek, DC Gruenert |
Cell & Tissue Research | 2012 |
Requirements for ion and solute transport, and pH regulation during enamel maturation
RS Lacruz, CE Smith, P Moffatt, EH Chang, TG Bromage, P Bringas, A Nanci, SK Baniwal, J Zabner, MJ Welsh, I Kurtz, ML Paine |
Journal of Cellular Physiology | 2012 |
Genetically modified pigs for biomedical research
Y Luo, L Lin, L Bolund, TG Jensen, CB Sørensen |
Journal of Inherited Metabolic Disease | 2012 |
Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung
AA Pezzulo, XX Tang, MJ Hoegger, MH Alaiwa, S Ramachandran, TO Moninger, PH Karp, CL Wohlford-Lenane, HP Haagsman, M Eijk, B Bánfi, AR Horswill, DA Stoltz, PB McCray, MJ Welsh, J Zabner |
Nature | 2012 |
Computer-aided analysis of airway trees in micro-CT scans of ex vivo porcine lung tissue
C Bauer, R Adam, DA Stoltz, RR Beichel |
Computerized Medical Imaging and Graphics | 2012 |
Generation of minipigs with targeted transgene insertion by recombinase-mediated cassette exchange (RMCE) and somatic cell nuclear transfer (SCNT)
JE Jakobsen, MG Johansen, M Schmidt, F Dagnæs-Hansen, K Dam, A Gunnarsson, Y Liu, PM Kragh, R Li, IE Holm, H Callesen, JG Mikkelsen, AL Nielsen, AL Jørgensen |
Transgenic Research | 2012 |
Completion of the swine genome will simplify the production of swine as a large animal biomedical model
EM Walters, E Wolf, JJ Whyte, J Mao, S Renner, H Nagashima, E Kobayashi, J Zhao, KD Wells, JK Critser, LK Riley, RS Prather |
BMC Medical Genomics | 2012 |
Lentiviral vector gene transfer to porcine airways
PL Sinn, AL Cooney, M Oakland, DE Dylla, TJ Wallen, AA Pezzulo, EH Chang, PB McCray |
Molecular Therapy — Nucleic Acids | 2012 |
Human leukocytes regulate ganglioside expression in cultured micro-pig aortic endothelial cells
JH Cho, JS Kim, MU Lim, HK Min, DH Kwak, JS Ryu, JT Lee, SU Kim, CH Kim, CH Kim, DB Koo, KT Chang, YK Choo |
Laboratory animal research | 2012 |
Potential of primary kidney cells for somatic cell nuclear transfer mediated transgenesis in pig
A Richter, M Kurome, B Kessler, V Zakhartchenko, N Klymiuk, H Nagashima, E Wolf, A Wuensch |
BMC Biotechnology | 2012 |
Simplified and versatile method for isolation of high-quality RNA from pancreas
M Griffin, M Abu-El-Haija, M Abu-El-Haija, T Rokhlina, A Uc |
BioTechniques | 2012 |
CGRP Induction in Cystic Fibrosis Airways Alters The Submucosal Gland Progenitor Cell Niche in Mice
Weiliang Xie, John T. Fisher, Thomas J. Lynch, Meihui Luo, Turan I.A. Evans, Traci L. Neff, Weihong Zhou, Yulong Zhang, Yi Ou, Nigel W. Bunnett, Andrew F. Russo, Michael J. Goodheart, Kalpaj R. Parekh, Xiaoming Liu, John F. Engelhardt |
Journal of Clinical Investigation | 2011 |
Swine Models of Cystic Fibrosis Reveal Male Reproductive Tract Phenotype at Birth1
F Pierucci-Alves, V Akoyev, JC 3rd, LH Wang, KS Janardhan, BD Schultz |
Biology of reproduction | 2011 |
Genetically manipulated mouse models of lung disease: potential and pitfalls
RM Baron, AJ Choi, CA Owen, AM Choi |
American journal of physiology. Lung cellular and molecular physiology | 2011 |
Cystic Fibrosis
MD Amaral, K Kunzelmann |
Cystic Fibrosis | 2011 |
Defective Fluid Secretion from Submucosal Glands of Nasal Turbinates from CFTR-/- and CFTRΔF508/ΔF508 Pigs
HJ Cho, NS Joo, JJ Wine, N Vij |
PloS one | 2011 |
The ΔF508 Mutation Causes CFTR Misprocessing and Cystic Fibrosis-Like Disease in Pigs
LS Ostedgaard, DK Meyerholz, JH Chen, AA Pezzulo, PH Karp, T Rokhlina, SE Ernst, RA Hanfland, LR Reznikov, PS Ludwig, MP Rogan, GJ Davis, CL Dohrn, C Wohlford-Lenane, PJ Taft, MV Rector, E Hornick, BS Nassar, M Samuel, Y Zhang, SS Richter, A Uc, J Shilyansky, RS Prather, PB McCray, J Zabner, MJ Welsh, DA Stoltz |
Science Translational Medicine | 2011 |
Genetic therapies for cystic fibrosis lung disease
PL Sinn, RM Anthony, PB McCray |
Human Molecular Genetics | 2011 |
Sequential targeting of CFTR by BAC vectors generates a novel pig model of cystic fibrosis
N Klymiuk, L Mundhenk, K Kraehe, A Wuensch, S Plog, D Emrich, MC Langenmayer, M Stehr, A Holzinger, C Kröner, A Richter, B Kessler, M Kurome, M Eddicks, H Nagashima, K Heinritzi, AD Gruber, E Wolf |
Journal of Molecular Medicine | 2011 |
New animal models of cystic fibrosis: what are they teaching us?
NW Keiser, JF Engelhardt |
Current Opinion in Pulmonary Medicine | 2011 |
Strategies for selection marker-free swine transgenesis using the Sleeping Beauty transposon system
DF Carlson, JR Garbe, W Tan, MJ Martin, JR Dobrinsky, PB Hackett, KJ Clark, SC Fahrenkrug |
Transgenic Research | 2011 |
Functional characterization of the ER stress induced X-box-binding protein-1 (Xbp-1) in the porcine system
JY Zhang, KS Lee, JS Kim, BS Song, DI Jin, DB Koo, K Yu |
BMC Molecular Biology | 2011 |
Genetic modifications of pigs for medicine and agriculture
JJ Whyte, RS Prather |
Molecular Reproduction and Development | 2011 |
An Evolved Adeno-associated Viral Variant Enhances Gene Delivery and Gene Targeting in Neural Stem Cells
JH Jang, JT Koerber, JS Kim, P Asuri, T Vazin, M Bartel, A Keung, I Kwon, KI Park, DV Schaffer |
Molecular Therapy | 2011 |
An update on targeted gene repair in mammalian cells: methods and mechanisms
NM Jensen, T Dalsgaard, M Jakobsen, RR Nielsen, CB Sørensen, L Bolund, TG Jensen |
Journal of Biomedical Science | 2011 |
An improved PCR strategy for fast screening of specific and random integrations in rAAV-mediated gene targeted cell clones
Y Luo, L Bolund, CB Sørensen |
BMC Research Notes | 2011 |
Efficient immunoglobulin gene disruption and targeted replacement in rabbit using zinc finger nucleases
T Flisikowska, IS Thorey, S Offner, F Ros, V Lifke, B Zeitler, O Rottmann, A Vincent, L Zhang, S Jenkins, H Niersbach, AJ Kind, PD Gregory, AE Schnieke, J Platzer |
PloS one | 2011 |
Efficient production of Fah-null heterozygote pigs by chimeric adeno-associated virus-mediated gene knockout and somatic cell nuclear transfer
RD Hickey, JB Lillegard, JE Fisher, TJ McKenzie, SE Hofherr, MJ Finegold, SL Nyberg, M Grompe |
Hepatology | 2011 |
Pig gene knockout by rAAV-mediated homologous recombination: comparison of BRCA1 gene knockout efficiency in Yucatan and Göttingen fibroblasts with slightly different target sequences
Y Luo, L Bolund, CB Sørensen |
Transgenic Research | 2011 |
Concentration of the antibacterial precursor thiocyanate in cystic fibrosis airway secretions
D Lorentzen, L Durairaj, AA Pezzulo, Y Nakano, J Launspach, DA Stoltz, G Zamba, PB McCray, J Zabner, MJ Welsh, WM Nauseef, B Bánfi |
Free radical biology & medicine | 2011 |
Endogenous surface expression of ΔF508-CFTR mediates cAMP-stimulated Cl − current in CFTR ΔF508/ΔF508 pig thyroid epithelial cells: porcine ΔF508-CFTR mediates cAMP-stimulated Cl − current
Y Li, S Ganta, P Fong |
Experimental Physiology | 2011 |
Comparison of potency between histone deacetylase inhibitors trichostatin A and valproic acid on enhancing in vitro development of porcine somatic cell nuclear transfer embryos
YJ Kim, KS Ahn, M Kim, H Shim |
In vitro cellular & developmental biology. Animal | 2011 |
Hyposecretion of Fluid from Tracheal Submucosal Glands of CFTR–deficient Pigs
Nam Soo Joo, Hyungju Cho, Monal Khansaheb, Jeffrey J. Wine |
Journal of Clinical Investigation | 2010 |
Pathology of Gastrointestinal Organs in a Porcine Model of Cystic Fibrosis
DK Meyerholz, DA Stoltz, AA Pezzulo, MJ Welsh |
The American Journal of Pathology | 2010 |
Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth
MP Rogan, LR Reznikov, AA Pezzulo, ND Gansemer, M Samuel, RS Prather, J Zabner, DC Fredericks, PB McCray, MJ Welsh, DA Stoltz |
Proceedings of the National Academy of Sciences | 2010 |
Respiratory Syncytial Virus Engineered To Express the Cystic Fibrosis Transmembrane Conductance Regulator Corrects the Bioelectric Phenotype of Human Cystic Fibrosis Airway Epithelium In Vitro
AR Kwilas, MA Yednak, L Zhang, R Liesman, PL Collins, RJ Pickles, ME Peeples |
Journal of virology | 2010 |
Cystic Fibrosis Pigs Develop Lung Disease and Exhibit Defective Bacterial Eradication at Birth
DA Stoltz, DK Meyerholz, AA Pezzulo, S Ramachandran, MP Rogan, GJ Davis, RA Hanfland, C Wohlford-Lenane, CL Dohrn, JA Bartlett, GA 4th, EH Chang, PJ Taft, PS Ludwig, M Estin, EE Hornick, JL Launspach, M Samuel, T Rokhlina, PH Karp, LS Ostedgaard, A Uc, TD Starner, AR Horswill, KA Brogden, RS Prather, SS Richter, J Shilyansky, PB McCray, J Zabner, MJ Welsh |
Science Translational Medicine | 2010 |
Sequencing and characterization of the porcine α-galactosidase A gene: towards the generation of a porcine model for Fabry disease
M Yoshimitsu, K Higuchi, X Fan, S Takao, JA Medin, C Tei, T Takenaka |
Molecular Biology Reports | 2010 |
Lentiviral Vectors and Cystic Fibrosis Gene Therapy
S Castellani, M Conese |
Viruses | 2010 |
Loss of Anion Transport without Increased Sodium Absorption Characterizes Newborn Porcine Cystic Fibrosis Airway Epithelia
JH Chen, DA Stoltz, PH Karp, SE Ernst, AA Pezzulo, TO Moninger, MV Rector, LR Reznikov, JL Launspach, K Chaloner, J Zabner, MJ Welsh |
Cell | 2010 |
Targeted disruption of the porcine immunoglobulin kappa light chain locus
J Ramsoondar, M Mendicino, C Phelps, T Vaught, S Ball, J Monahan, S Chen, A Dandro, J Boone, P Jobst, A Vance, N Wertz, I Polejaeva, J Butler, Y Dai, D Ayares, K Wells |
Transgenic Research | 2010 |
Generation of antibody- and B cell-deficient pigs by targeted disruption of the J-region gene segment of the heavy chain locus
M Mendicino, J Ramsoondar, C Phelps, T Vaught, S Ball, T LeRoith, J Monahan, S Chen, A Dandro, J Boone, P Jobst, A Vance, N Wertz, Z Bergman, XZ Sun, I Polejaeva, J Butler, Y Dai, D Ayares, K Wells |
Transgenic Research | 2010 |
Transgenic pigs as models for translational biomedical research
B Aigner, S Renner, B Kessler, N Klymiuk, M Kurome, A Wünsch, E Wolf |
Journal of Molecular Medicine | 2010 |
RNA Interference in Pigs: Comparison of RNAi Test Systems and Expression Vectors
C Merkl, S Leuchs, A Saalfrank, A Kind, A Schnieke |
Molecular Biotechnology | 2010 |
High efficiency of BRCA1 knockout using rAAV-mediated gene targeting: developing a pig model for breast cancer
Y Luo, J Li, Y Liu, L Lin, Y Du, S Li, H Yang, G Vajta, H Callesen, L Bolund, CB Sørensen |
Transgenic Research | 2010 |
Altered ion transport by thyroid epithelia from CFTR −/− pigs suggests mechanisms for hypothyroidism in cystic fibrosis: Function of CFTR in thyroid of novel porcine model of cystic fibrosis
H Li, S Ganta, P Fong |
Experimental Physiology | 2010 |
Potential of anti-inflammatory treatment for cystic fibrosis lung disease.
Taylor-Cousar JL, Von Kessel KA, Young R, Nichols DP |
Journal of inflammation research | 2010 |
Substance P stimulates human airway submucosal gland secretion mainly via a CFTR-dependent process
1. Jae Young Choi 2. Monal Sonecha 3. Nam Soo Joo 4. Mauri E. Krouse 5. Robert C. Robbins 6. David Weill 7. Jeffrey J. Wine. |
Journal of Clinical Investigation | 2009 |
Hemizygous minipigs produced by random gene insertion and handmade cloning express the Alzheimer's disease-causing dominant mutation APPsw
PM Kragh, AL Nielsen, J Li, Y Du, L Lin, M Schmidt, IB Bøgh, IE Holm, JE Jakobsen, MG Johansen, S Purup, L Bolund, G Vajta, AL Jørgensen |
Transgenic Research | 2009 |
Pulmonary surfactant: an immunological perspective
ZC Chroneos, Z Sever-Chroneos, VL Shepherd |
Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacology | 2009 |
Progress and prospects: techniques for site-directed mutagenesis in animal models
Z Yan, X Sun, JF Engelhardt |
Gene Therapy | 2009 |
Indexing TNF-αgene expression using a gene-targeted reporter cell line
Z Yan, D Lei-Butters, JF Engelhardt, GH Leno |
BMC Biology | 2009 |
Optimization of square-wave electroporation for transfection of porcine fetal fibroblasts
JW Ross, JJ Whyte, J Zhao, M Samuel, KD Wells, RS Prather |
Transgenic Research | 2009 |
Genomic, tissue expression, and protein characterization of pCLCA1, a putative modulator of cystic fibrosis in the pig
S Plog, L Mundhenk, N Klymiuk, AD Gruber |
The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society | 2009 |
Significant improvement in cloning efficiency of an inbred miniature pig by histone deacetylase inhibitor treatment after somatic cell nuclear transfer
J Zhao, JW Ross, Y Hao, LD Spate, EM Walters, MS Samuel, A Rieke, CN Murphy, RS Prather |
Biology of reproduction | 2009 |
Highlights of a workshop to discuss targeting inflammation in cystic fibrosis
KH Banner, HD Jonge, S Elborn, E Growcott, E Gulbins, M Konstan, R Moss, C Poll, SH Randell, AG Rossi, L Thomas, D Waltz |
Journal of Cystic Fibrosis | 2009 |
Generation of transgenic non-human primates with germline transmission
E Sasaki, H Suemizu, A Shimada, K Hanazawa, R Oiwa, M Kamioka, I Tomioka, Y Sotomaru, R Hirakawa, T Eto, S Shiozawa, T Maeda, M Ito, R Ito, C Kito, C Yagihashi, K Kawai, H Miyoshi, Y Tanioka, N Tamaoki, S Habu, H Okano, T Nomura |
Nature | 2009 |
Genetic manipulation in pigs.
Sachs DH, Galli C |
Current Opinion in Organ Transplantation | 2009 |
AAV Targeted Disruption of the CFTR Gene in Cloned Ferrets
Xingshen Sun; Ziying Yan; Yaling Yi; Ziyi Li; Diana Lei; Christopher S. Rogers; Juan Chen; Michael J. Welsh; Gregory H. Leno; and John F. Engelhardt |
Journal of Clinical Investigation | 2008 |
Analysis of Adeno-associated Virus Progenitor Cell Transduction in Mouse Lung
X Liu, M Luo, C Guo, Z Yan, Y Wang, DC Lei-Butters, JF Engelhardt |
Molecular Therapy | 2008 |
CFTR and defective endocytosis: new insights in the renal phenotype of cystic fibrosis
F Jouret, O Devuyst |
Pflügers Archiv - European Journal of Physiology | 2008 |
The porcine lung as a potential model for cystic fibrosis
CS Rogers, WM Abraham, KA Brogden, JF Engelhardt, JT Fisher, PB McCray, G McLennan, DK Meyerholz, E Namati, LS Ostedgaard, RS Prather, JR Sabater, DA Stoltz, J Zabner, MJ Welsh |
American journal of physiology. Lung cellular and molecular physiology | 2008 |
Transgene expression of enhanced green fluorescent protein in cloned rabbits generated from in vitro-transfected adult fibroblasts
S Li, Y Guo, J Shi, C Yin, F Xing, L Xu, C Zhang, T Liu, Y Li, H Li, L Du, X Chen |
Transgenic Research | 2008 |
Disruption of the CFTR Gene Produces a Model of Cystic Fibrosis in Newborn Pigs
CS Rogers, DA Stoltz, DK Meyerholz, LS Ostedgaard, T Rokhlina, PJ Taft, MP Rogan, AA Pezzulo, PH Karp, OA Itani, AC Kabel, CL Wohlford-Lenane, GJ Davis, RA Hanfland, TL Smith, M Samuel, D Wax, CN Murphy, A Rieke, K Whitworth, A Uc, TD Starner, KA Brogden, J Shilyansky, PB McCray, J Zabner, RS Prather, MJ Welsh |
Science | 2008 |
Production of Prnp −/− goats by gene targeting in adult fibroblasts
C Zhu, B Li, G Yu, J Chen, H Yu, J Chen, X Xu, Y Wu, A Zhang, G Cheng |
Transgenic Research | 2008 |