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Bithiazole correctors rescue CFTR mutants by two different mechanisms.


ABSTRACT: Better correctors are needed to repair cystic fibrosis transmembrane conductance regulator (CFTR) processing mutants that cause cystic fibrosis. Determining where the correctors bind to CFTR would aid in the development of new correctors. A recent study reported that the second nucleotide-binding domain (NBD2) was involved in binding of bithiazole correctors. Here, we show that bithiazole correctors could also rescue CFTR mutants that lacked NBD2. These results suggest that bithiazoles rescue CFTR mutants by two different mechanisms.

SUBMITTER: Loo TW 

PROVIDER: S-EPMC3737597 | biostudies-literature | 2013 Aug

REPOSITORIES: biostudies-literature

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Bithiazole correctors rescue CFTR mutants by two different mechanisms.

Loo Tip W TW   Bartlett M Claire MC   Clarke David M DM  

Biochemistry 20130722 31


Better correctors are needed to repair cystic fibrosis transmembrane conductance regulator (CFTR) processing mutants that cause cystic fibrosis. Determining where the correctors bind to CFTR would aid in the development of new correctors. A recent study reported that the second nucleotide-binding domain (NBD2) was involved in binding of bithiazole correctors. Here, we show that bithiazole correctors could also rescue CFTR mutants that lacked NBD2. These results suggest that bithiazoles rescue CF  ...[more]

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