The objective of the Mouse Genetics Core is to establish and manage a resource that will generate and provide mouse models for the studies of cystic fibrosis under this NIH SCOR Program Grant. Specifically, and as described in detail in the individual proposals and below, the new mouse lines to be generated under his Core include: Project 1 (K.L. Kirk)....(Tg) FABP-p11 (WT) and (Tg) FABP-p11 (Mutant) Project 2 (D.J. Benos).....(Tg) CLCA3 and (KO) CLCA3 Project 3 (D.M. Bedwell).... (KI) CFTR-G542 (UGA), (KI) CFTR-G542 (UAG) and (KI) CFTR-G542 UAA). The mouse lines will be generated by oocyte microinjection (Tg) FABP-p1 1 (WT), (Tg) FABP-p1 1 (Mutant) and (Tg) CLCA3) and by embryonic stem cell gene targeting (KO) CLCA3, (KI) CFTR-G542 (UGA), (KI) CFTR-G542 (UAG) and (KI) CFTR-G542 (UAA) technologies, as described in the Research Plan. Confirmation of fidelity to he genomic integration or modification, and the desired expression will be performed under the auspices of the 'individual projects. The Mouse Genetics Core will also establish breeding colonies of the mice described by the individual projects of this NIH SCOR Program, genotype and provide them to the investigators as needed. In addition to generating and propagating the new mouse lines described above, the Mouse Genetics Core will maintain colonies and provide to investigators several established mouse lines relevant to the outlined studies. As described in detail in the individual projects and summarized in the Research Plan, the mouse lines to be maintained under this Core.

Agency
National Institute of Health (NIH)
Institute
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Type
Specialized Center (P50)
Project #
2P50DK053090-06
Application #
6583539
Study Section
Special Emphasis Panel (ZDK1)
Project Start
2002-09-01
Project End
2007-08-31
Budget Start
Budget End
Support Year
6
Fiscal Year
2002
Total Cost
Indirect Cost
Name
University of Alabama Birmingham
Department
Type
DUNS #
004514360
City
Birmingham
State
AL
Country
United States
Zip Code
35294
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Du, Ming; Liu, Xiaoli; Welch, Ellen M et al. (2008) PTC124 is an orally bioavailable compound that promotes suppression of the human CFTR-G542X nonsense allele in a CF mouse model. Proc Natl Acad Sci U S A 105:2064-9
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Gaggar, Amit; Li, Yao; Weathington, Nathaniel et al. (2007) Matrix metalloprotease-9 dysregulation in lower airway secretions of cystic fibrosis patients. Am J Physiol Lung Cell Mol Physiol 293:L96-L104
Benos, Dale J; Bashari, Edlira; Chaves, Jose M et al. (2007) The ups and downs of peer review. Adv Physiol Educ 31:145-52
Kellermayer, Richard; Szigeti, Reka; Keeling, Kim M et al. (2006) Aminoglycosides as potential pharmacogenetic agents in the treatment of Hailey-Hailey disease. J Invest Dermatol 126:229-31
Su, Xuefeng; Li, Qingnan; Shrestha, Kedar et al. (2006) Interregulation of proton-gated Na(+) channel 3 and cystic fibrosis transmembrane conductance regulator. J Biol Chem 281:36960-8
Du, Ming; Keeling, Kim M; Fan, Liming et al. (2006) Clinical doses of amikacin provide more effective suppression of the human CFTR-G542X stop mutation than gentamicin in a transgenic CF mouse model. J Mol Med 84:573-82

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