Effect of deleting the R domain on CFTR-generated chloride channels

D. P RICH; R. J GREGORY; M. P ANDERSON; P MANAVALAN; A. E SMITH; M. J WELSH

doi:10.1126/science.1712985

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Effect of deleting the R domain on CFTR-generated chloride channels

Journal article

Peer reviewed

Effect of deleting the R domain on CFTR-generated chloride channels

D. P RICH, R. J GREGORY, M. P ANDERSON, P MANAVALAN, A. E SMITH and M. J WELSH

Science (American Association for the Advancement of Science), Vol.253(5016), pp.205-207

1991

DOI: 10.1126/science.1712985

PMID: 1712985

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Abstract

The cystic fibrosis transmembrane conductance regulator (CFTR), which forms adenosine 3′,5′-monophosphate (cAMP)-regulated chloride channels, is defective in patients with cystic fibrosis. This protein contains two putative nucleotide binding domains (NBD1 and NBD2) and an R domain. CFTR in which the R domain was deleted (CFTRΔR) conducted chloride independently of the presence of cAMP. However, sites within CFTR other than those deleted also respond to cAMP, because the chloride current of CFTRΔR increased further in response to cAMP stimulation. In addition, deletion of the R domain suppressed the inactivating effect of a mutation in NBD2 (but not NBD1), a result which suggests that NBD2 interacts with the channel through the R domain.

Gastroenterology. Liver. Pancreas. Abdomen

Biological and medical sciences

Medical sciences

Liver. Biliary tract. Portal circulation. Exocrine pancreas

Other diseases. Semiology

Details

Title: Subtitle: Effect of deleting the R domain on CFTR-generated chloride channels
Creators: D. P RICH - Univ. Iowa coll. medicine, Howard Hughes medical inst., dep. internal medicine physiology, Iowa city IA 52242, United States
R. J GREGORY - Univ. Iowa coll. medicine, Howard Hughes medical inst., dep. internal medicine physiology, Iowa city IA 52242, United States
M. P ANDERSON - Univ. Iowa coll. medicine, Howard Hughes medical inst., dep. internal medicine physiology, Iowa city IA 52242, United States
P MANAVALAN - Univ. Iowa coll. medicine, Howard Hughes medical inst., dep. internal medicine physiology, Iowa city IA 52242, United States
A. E SMITH - Univ. Iowa coll. medicine, Howard Hughes medical inst., dep. internal medicine physiology, Iowa city IA 52242, United States
M. J WELSH - Univ. Iowa coll. medicine, Howard Hughes medical inst., dep. internal medicine physiology, Iowa city IA 52242, United States
Resource Type: Journal article
Publication Details: Science (American Association for the Advancement of Science), Vol.253(5016), pp.205-207
Publisher: American Association for the Advancement of Science; Washington, DC
DOI: 10.1126/science.1712985
PMID: 1712985
ISSN: 0036-8075
eISSN: 1095-9203
Language: English
Date published: 1991
Academic Unit: Neurology; Molecular Physiology and Biophysics; Neurosurgery; Internal Medicine
Record Identifier: 9984020719702771

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