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• W2138836503 endingPage "1946" @default.
• W2138836503 startingPage "1933" @default.
• W2138836503 abstract "More than 130 different mutations in the gap junction integral plasma membrane protein connexin32 (Cx32) have been linked to the human peripheral neuropathy X-linked Charcot-Marie-Tooth disease (CMTX). How these various mutants are processed by the cell and the mechanism(s) by which they cause CMTX are unknown. To address these issues, we have studied the intracellular transport, assembly, and degradation of three CMTX-linked Cx32 mutants stably expressed in PC12 cells. Each mutant had a distinct fate: E208K Cx32 appeared to be retained in the endoplasmic reticulum (ER), whereas both the E186K and R142W mutants were transported to perinuclear compartments from which they trafficked either to lysosomes (R142W Cx32) or back to the ER (E186K Cx32). Despite these differences, each mutant was soluble in nonionic detergent but unable to assemble into homomeric connexons. Degradation of both mutant and wild-type connexins was rapid (t(1/2) < 3 h) and took place at least in part in the ER by a process sensitive to proteasome inhibitors. The mutants studied are therefore unlikely to cause disease by accumulating in degradation-resistant aggregates but instead are efficiently cleared from the cell by quality control processes that prevent abnormal connexin molecules from traversing the secretory pathway." @default.
• W2138836503 created "2016-06-24" @default.
• W2138836503 creator A5040065524 @default.
• W2138836503 creator A5071984892 @default.
• W2138836503 creator A5077324643 @default.
• W2138836503 date "2000-06-01" @default.
• W2138836503 modified "2023-10-14" @default.
• W2138836503 title "Intracellular Transport, Assembly, and Degradation of Wild-Type and Disease-linked Mutant Gap Junction Proteins" @default.
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• W2138836503 doi "https://doi.org/10.1091/mbc.11.6.1933" @default.
• W2138836503 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/14894" @default.
• W2138836503 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/10848620" @default.
• W2138836503 hasPublicationYear "2000" @default.
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