Abstract

Since the identification of the gene responsible for cystic fibrosis (CF), the cystic fibrosis transmembrane conductance regulator (CFTR), the availability and use of molecular biologic techniques has become essential in the study of the molecular defect in this disease. This SCOR is focused on the study of the structure and function of CFTR and upon other ion channels functionally associated with epithelial secretion. Each of the Research Projects will utilize molecular biologic techniques. The Core will provide reagents such as expression vectors containing either wild-type or mutant forms of CFTR, assistance in the subcloning of individual channels such as the cGMP gated Na+ channel into expression vectors, facilities and expertise for cDNA sequencing, site- directed mutagenesis and cell transfection.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Type
Specialized Center (P50)
Project #
1P50DK048977-01
Application #
3754792
Study Section
Project Start
Project End
Budget Start
Budget End
Support Year
1
Fiscal Year
1994
Total Cost
Indirect Cost

  Institution

Name
Johns Hopkins University
Department
Type
DUNS #
045911138
City
Baltimore
State
MD
Country
United States
Zip Code
21218

  Publications

Guggino, William B (2004) The cystic fibrosis transmembrane regulator forms macromolecular complexes with PDZ domain scaffold proteins. Proc Am Thorac Soc 1:28-32
Ketchum, Christian J; Rajendrakumar, Garnepudi V; Maloney, Peter C (2004) Characterization of the adenosinetriphosphatase and transport activities of purified cystic fibrosis transmembrane conductance regulator. Biochemistry 43:1045-53
Cheng, Jie; Wang, Hua; Guggino, William B (2004) Modulation of mature cystic fibrosis transmembrane regulator protein by the PDZ domain protein CAL. J Biol Chem 279:1892-8
Swiatecka-Urban, Agnieszka; Duhaime, Marc; Coutermarsh, Bonita et al. (2002) PDZ domain interaction controls the endocytic recycling of the cystic fibrosis transmembrane conductance regulator. J Biol Chem 277:40099-105
Cheng, Jie; Moyer, Bryan D; Milewski, Michal et al. (2002) A Golgi-associated PDZ domain protein modulates cystic fibrosis transmembrane regulator plasma membrane expression. J Biol Chem 277:3520-9
Ketchum, Christian J; Yue, Hongwen; Alessi, Karen A et al. (2002) Intracellular cysteines of the cystic fibrosis transmembrane conductance regulator (CFTR) modulate channel gating. Cell Physiol Biochem 12:1-8
Norlin, A; Lu, L N; Guggino, S E et al. (2001) Contribution of amiloride-insensitive pathways to alveolar fluid clearance in adult rats. J Appl Physiol 90:1489-96
Ketchum, C J; Schmidt, W K; Rajendrakumar, G V et al. (2001) The yeast a-factor transporter Ste6p, a member of the ABC superfamily, couples ATP hydrolysis to pheromone export. J Biol Chem 276:29007-11
Mickle, J E; Milewski, M I; Macek Jr, M et al. (2000) Effects of cystic fibrosis and congenital bilateral absence of the vas deferens-associated mutations on cystic fibrosis transmembrane conductance regulator-mediated regulation of separate channels. Am J Hum Genet 66:1485-95
Moyer, B D; Duhaime, M; Shaw, C et al. (2000) The PDZ-interacting domain of cystic fibrosis transmembrane conductance regulator is required for functional expression in the apical plasma membrane. J Biol Chem 275:27069-74

Showing the most recent 10 out of 39 publications