The CFTR-targeted pigs produced by Exemplar Genetics are discussed or studied in the following publications:
Requirements for ion and solute transport, and pH regulation, during enamel maturation.
Lacruz RS, Smith CE, Moffatt P, Chang EH, Bromage TG, Bringas P Jr, Nanci A, Baniwal SK, Zabner J, Welsh MJ, Kurtz I, Paine ML. Center for Craniofacial Molecular Biology, Herman Ostrow School of Dentistry, 2250 Alcazar Street, CSA Room #103, Los Angeles, CA 90033, USA. PMID: 21732355 [PubMed - as supplied by publisher]
Efficient production of Fah-null heterozygote pigs by chimeric adeno-associated virus-mediated gene knockout and somatic cell nuclear transfer.
Hickey RD, Lillegard JB, Fisher JE, McKenzie TJ, Hofherr SE, Finegold MJ, Nyberg SL, Grompe M. Papé Family Pediatric Research Institute & Oregon Stem Cell Center, Oregon Health & Sciences University, Portland, Oregon 97239, USA; Department of Molecular & Medical Genetics, Oregon Health & Sciences University, Portland, Oregon 97239, USA. PMID: 21674562 [PubMed - as supplied by publisher]
Enamel Pathology Resulting from Loss of Function in the Cystic Fibrosis Transmembrane Conductance Regulator in a Porcine Animal Model.
Chang EH, Lacruz RS, Bromage TG, Bringas P Jr, Welsh MJ, Zabner J, Paine ML. Department of Otolaryngology, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA.
The {Delta}F508 Mutation Causes CFTR Misprocessing and Cystic Fibrosis-Like Disease in Pigs.
Ostedgaard LS, Meyerholz DK, Chen JH, Pezzulo AA, Karp PH, Rokhlina T, Ernst SE, Hanfland RA, Reznikov LR, Ludwig PS, Rogan MP, Davis GJ, Dohrn CL, Wohlford-Lenane C, Taft PJ, Rector MV, Hornick E, Nassar BS, Samuel M, Zhang Y, Richter SS, Uc A, Shilyansky J, Prather RS, McCray PB Jr, Zabner J, Welsh MJ, Stoltz DA.
Sci Transl Med. 2011 Mar 16;3(74):74ra24.
PMID: 21411740 [PubMed - in process]
Cystic fibrosis: an-ion transport issue?
Pier G, Prince A, Cantin A.
Nat Med. 2011 Feb;17(2):166-7. No abstract available.
PMID: 21297610 [PubMed - indexed for MEDLINE]
Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia.
Chen JH, Stoltz DA, Karp PH, Ernst SE, Pezzulo AA, Moninger TO, Rector MV, Reznikov LR, Launspach JL, Chaloner K, Zabner J, Welsh MJ.
Cell. 2010 Dec 10;143(6):911-23.
PMID: 21145458 [PubMed - indexed for MEDLINE]
Thyroid glands from pigs with cystic fibrosis, old issues new ways.
Scholte BJ.
Exp Physiol. 2010 Dec;95(12):1131. No abstract available.
PMID: 21097980 [PubMed - indexed for MEDLINE]
Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth.
Rogan MP, Reznikov LR, Pezzulo AA, Gansemer ND, Samuel M, Prather RS, Zabner J, Fredericks DC, McCray PB Jr, Welsh MJ, Stoltz DA.
Proc Natl Acad Sci U S A. 2010 Nov 23;107(47):20571-5. Epub 2010 Nov 8.
PMID: 21059918 [PubMed - indexed for MEDLINE]
Hyposecretion of fluid from tracheal submucosal glands of CFTR-deficient pigs.
Joo NS, Cho HJ, Khansaheb M, Wine JJ.
J Clin Invest. 2010 Sep 1;120(9):3161-6. doi: 10.1172/JCI43466. Epub 2010 Aug 25.
PMID: 20739758 [PubMed - indexed for MEDLINE]
cAMP-activated Ca2+ signaling is required for CFTR-mediated serous cell fluid secretion in porcine and human airways.
Lee RJ, Foskett JK.
J Clin Invest. 2010 Sep 1;120(9):3137-48. doi: 10.1172/JCI42992. Epub 2010 Aug 25.
PMID: 20739756 [PubMed - indexed for MEDLINE]
Transgenic animals may help resolve a sticky situation in cystic fibrosis.
Widdicombe JH.
J Clin Invest. 2010 Sep 1;120(9):3093-6. doi: 10.1172/JCI44235. Epub 2010 Aug 25.
PMID: 20739746 [PubMed - indexed for MEDLINE]
Altered ion transport by thyroid epithelia from CFTR(-/-) pigs suggests mechanisms for hypothyroidism in cystic fibrosis.
Li H, Ganta S, Fong P.
Exp Physiol. 2010 Dec;95(12):1132-44. Epub 2010 Aug 20.
PMID: 20729267 [PubMed - indexed for MEDLINE]
Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children.
Meyerholz DK, Stoltz DA, Namati E, Ramachandran S, Pezzulo AA, Smith AR, Rector MV, Suter MJ, Kao S, McLennan G, Tearney GJ, Zabner J, McCray PB Jr, Welsh MJ.
Am J Respir Crit Care Med. 2010 Nov 15;182(10):1251-61. Epub 2010 Jul 9.
Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth.
Stoltz DA, Meyerholz DK, Pezzulo AA, Ramachandran S, Rogan MP, Davis GJ, Hanfland RA, Wohlford-Lenane C, Dohrn CL, Bartlett JA, Nelson GA 4th, Chang EH, Taft PJ, Ludwig PS, Estin M, Hornick EE, Launspach JL, Samuel M, Rokhlina T, Karp PH, Ostedgaard LS, Uc A, Starner TD, Horswill AR, Brogden KA, Prather RS, Richter SS, Shilyansky J, McCray PB Jr, Zabner J, Welsh MJ.
Sci Transl Med. 2010 Apr 28;2(29):29ra31.
PMID: 20427821 [PubMed - indexed for MEDLINE]
The development of lung disease in cystic fibrosis pigs.
Wine JJ.
Sci Transl Med. 2010 Apr 28;2(29):29ps20.
PMID: 20427819 [PubMed - indexed for MEDLINE]
Pathology of gastrointestinal organs in a porcine model of cystic fibrosis.
Meyerholz DK, Stoltz DA, Pezzulo AA, Welsh MJ.
Am J Pathol. 2010 Mar;176(3):1377-89. Epub 2010 Jan 28.
PMID: 20110417 [PubMed - indexed for MEDLINE]
Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs.
Rogers CS, Stoltz DA, Meyerholz DK, Ostedgaard LS, Rokhlina T, Taft PJ, Rogan MP, Pezzulo AA, Karp PH, Itani OA, Kabel AC, Wohlford-Lenane CL, Davis GJ, Hanfland RA, Smith TL, Samuel M, Wax D, Murphy CN, Rieke A, Whitworth K, Uc A, Starner TD, Brogden KA, Shilyansky J, McCray PB Jr, Zabner J, Prather RS, Welsh MJ.
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