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Cystic fibrosis arises from the misfolding and premature degradation of CFTRΔF508, a Cl − ion channel with a single amino acid deletion. Yet, the quality-control machinery that selects CFTRΔF508 for degradation and the mechanism for its misfolding are not well defined. We identified an ER membrane-associated ubiquitin ligase complex containing the E3 RMA1, the E2 Ubc6e, and Derlin-1 that cooperates...
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