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Sec63 and Xbp1 regulate IRE1α activity and polycystic disease severity

5 Apr 2015

Abstract excerpt

The HSP40 cochaperone SEC63 is associated with the SEC61 translocon complex in the ER. Mutations in the gene encoding SEC63 cause polycystic liver disease in humans; however, it is not clear how altered SEC63 influences disease manifestations. In mice, loss of SEC63 induces cyst formation both in liver and kidney as the result of reduced polycystin-1 (PC1). Here we report that inactivation of SEC63 induces an...

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PMID: 25844898
DOI: 10.1172/jci78863

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