Abstract

Three investigators within the overall SCOR proposal require the use of cystic fibrosis mice for their individual components. Dr. Bedwell (Project 1) intends experiments with a G542X CF mouse, in order to test whether aminoglycoside antibiotics can activate read-through of this truncated CFTR in vivo. Dr. Benos (Project 3) intends to develop a transgenic mouse possessing the Ca2+ - activated C1- channel (CaCC) under the regulatory control of the fatty acid binding protein promoter in order to determine whether it is possible to rescue cystic fibrosis mice by activating an alternate C1- secretory pathway. Dr. Sorscher (Project 4) will use the CF Animal Core facility for studies of a delta F508 maturational agent (DMSO) in cystic fibrosis mice. The CF Animal Core is intended to expand and genotype colonies of cystic fibrosis mice and to assist SCOR investigators with development of these two new strains (G542X, CaCC) of CF mice. The Core will also assist with nasal potential difference (PD) measurements on CF and normal mice in order to test interventions designed to correct the CF defect. While the primary responsibility of the CF Animal Core will be to assist SCOR investigators, many other faculty on the UAB campus will benefit from the existence of a core facility designated to develop and breed CF mice.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Type
Specialized Center (P50)
Project #
5P50DK053090-05
Application #
6504036
Study Section
Project Start
2001-09-01
Project End
2002-08-31
Budget Start
Budget End
Support Year
5
Fiscal Year
2001
Total Cost
Indirect Cost

  Institution

Name
University of Alabama Birmingham
Department
Type
DUNS #
004514360
City
Birmingham
State
AL
Country
United States
Zip Code
35294

  Publications

Du, Ming; Keeling, Kim M; Fan, Liming et al. (2009) Poly-L-aspartic acid enhances and prolongs gentamicin-mediated suppression of the CFTR-G542X mutation in a cystic fibrosis mouse model. J Biol Chem 284:6885-92
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
Guimbellot, Jennifer S; Fortenberry, James A; Siegal, Gene P et al. (2008) Role of oxygen availability in CFTR expression and function. Am J Respir Cell Mol Biol 39:514-21
Berdiev, Bakhrom K; Cormet-Boyaka, Estelle; Tousson, Albert et al. (2007) Molecular proximity of cystic fibrosis transmembrane conductance regulator and epithelial sodium channel assessed by fluorescence resonance energy transfer. J Biol Chem 282:36481-8
Rowe, Steven M; Varga, Karoly; Rab, Andras et al. (2007) Restoration of W1282X CFTR activity by enhanced expression. Am J Respir Cell Mol Biol 37:347-56
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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