PMID: 23784545

Cai Z, Li H, Chen JH, Sheppard DN
Acute inhibition of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel by thyroid hormones involves multiple mechanisms.
Am J Physiol Cell Physiol. 2013 Oct 15;305(8):C817-28. doi: 10.1152/ajpcell.00052.2013. Epub 2013 Jun 19., [PubMed]
Sentences
No. Mutations Sentence Comment
232 ABCC7 p.Gly551Asp
X
ABCC7 p.Gly551Asp 23784545:232:127
status: NEW
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By contrast, D&#e9;rand et al. (16) demonstrated that allosteric inhibition of CFTR by genistein is abolished by the CF mutant G551D-CFTR, which affects a key residue in ATP-binding site 2, the site of rapid ATP hydrolysis in the ATP-driven NBD dimerization model of CFTR channel gating (60, 61). Login to comment
251 ABCC7 p.Arg347Glu
X
ABCC7 p.Arg347Glu 23784545:251:125
status: NEW
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ABCC7 p.Arg352Glu
X
ABCC7 p.Arg352Glu 23784545:251:146
status: NEW
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ABCC7 p.Arg334Cys
X
ABCC7 p.Arg334Cys 23784545:251:108
status: NEW
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The occurrence of subconductance states is more frequent in some site-directed mutations in the MSDs [e.g., R334C-CFTR (70), R347E-CFTR (11), and R352E-CFTR (13)], while wild-type murine CFTR resides for prolonged periods in a minuscule subconductance state and only transitions infrequently to the full open-state (37). Login to comment
257 ABCC7 p.Arg352Cys
X
ABCC7 p.Arg352Cys 23784545:257:62
status: NEW
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Recognizing that sojourns to subconductance states in Cysless-R352C-CFTR mimic the behavior of wild-type CFTR blocked by MOPS, Jih et al. (33) developed the energetic coupling model of CFTR channel gating (for review, see Ref. 32). Login to comment