ABCMdb

PMID: 18716059

Rosser MF, Grove DE, Chen L, Cyr DM
Assembly and misassembly of cystic fibrosis transmembrane conductance regulator: folding defects caused by deletion of F508 occur before and after the calnexin-dependent association of membrane spanning domain (MSD) 1 and MSD2.
Mol Biol Cell. 2008 Nov;19(11):4570-9. Epub 2008 Aug 20., [PubMed]

Sentences

No. Mutations Sentence Comment

233 ABCC7 p.Asn1303Lys We next compared the trypsin proteolysis patterns of misfolded CFTR from CAS-treated cells with the patterns observed from CFTR⌬F508, CFTRG91R, and CFTR N1303K, which contain mutations localized to the NBD1, MSD1, and NBD2 domains, respectively (Osborne et al., 1992; Xiong et al., 1997). Login to comment 235 ABCC7 p.Asn1303Lys In contrast, mutation of N1303K in NBD2 did not drastically affect the stability of CFTR N-terminal fragments. Login to comment 239 ABCC7 p.Gly91Arg Yet, the fact that the G91R mutation, and to a lesser extent, inhibition of calnexin function also hinder NBD2 folding suggests that general disruption of MSD assembly prevents proper folding of NBD2. Login to comment