Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
نویسندگان
چکیده
منابع مشابه
Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
The molecular components of the quality control system that rapidly degrades abnormal membrane and secretory proteins have not been identified. The cystic fibrosis transmembrane conductance regulator (CFTR) is an integral membrane protein to which this quality control is stringently applied; approximately 75% of the wild-type precursor and 100% of the delta F508 CFTR variant found in most CF pa...
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Most cases of cystic fibrosis are caused by mutations that interfere with the biosynthetic folding of the cystic fibrosis transmembrane conductance regulator (CFTR), leading to the rapid degradation of CFTR molecules that have not matured beyond the endoplasmic reticulum (ER). The mechanism by which integral membrane proteins including CFTR are recognized and targeted for ER degradation and the...
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ژورنال
عنوان ژورنال: Cell
سال: 1995
ISSN: 0092-8674
DOI: 10.1016/0092-8674(95)90241-4