ABCMdb

PMID: 10851114

McCarty NA
Permeation through the CFTR chloride channel.
J Exp Biol. 2000 Jul;203(Pt 13):1947-62., [PubMed]

Sentences

No. Mutations Sentence Comment

169 ABCC7 p.Arg347Asp Mutation R347D at the putative cytoplasmic end of TM6 reduced the affinity for DIDS. Login to comment 170 ABCC7 p.Arg347Asp However, R347D has recently been shown to grossly perturb the conformation of the pore by disruption of a salt bridge (Cotten and Welsh, 1999). Login to comment 276 ABCC7 p.Arg334Trp ABCC7 p.Arg334Trp ABCC7 p.Arg347Pro ABCC7 p.Arg347Pro Here, it is believed that K335 and R347 influence selectivity and Cl-conductance (Tabcharani et al., 1997): R347 confers anomalous mole-fraction effects and protocol-dependent block by I- (Tabcharani et al., 1997); cysteine substitutions for several residues interact with SH-modifying reagents as if they face the pore (Cheung and Akabas, 1996); T338 and T339 together control the permeability of the channel to polyatomic anions as if they contribute to a narrow region (Linsdell et al., 1997b, 1998); disease-associated mutations (R334W and R347P) alter the kinetics and conductance of single channels (Sheppard et al., 1993); and the anion/cation selectivity filter is formed by T351, R352 and Q353 (Cheung and Akabas, 1997; Guinamard and Akabas, 1999). Login to comment