ABCMdb

PMID: 16901789

Younger JM, Chen L, Ren HY, Rosser MF, Turnbull EL, Fan CY, Patterson C, Cyr DM
Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulator.
Cell. 2006 Aug 11;126(3):571-82., [PubMed]

Sentences

No. Mutations Sentence Comment

207 ABCC7 p.Gly91Arg Hence, we compared the ability of RMA1 and CHIP to sense the folding defect caused by the G91R mutation in transmembrane helix I, which prevents proper assembly of CFTR (Xiong et al., 1997). Login to comment 208 ABCC7 p.Gly91Arg Indeed, CFTR1162X G91R was found to be sensitive to elevation of RMA1 activity, but not to CHIP. Login to comment 221 ABCC7 p.Gly91Arg First, the elevation of RMA1 activity drives the degradation of CFTR biogenic intermediates that have the G91R mutation in MSDI, which is degraded by ERAD due to defects in MSD assembly (Xiong et al., 1997). Login to comment