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. 2008 Aug 11;182(3):531–542. doi: 10.1083/jcb.200711151

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© 2008 Ju et al.

This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see http://www.jcb.org/misc/terms.shtml). After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 3.0 Unported license, as described at http://creativecommons.org/licenses/by-nc-sa/3.0/).

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Figure 8. — Working model for Cosmc function. Human Cosmc is localized predominantly in the ER where it interacts with the nascent polypeptide of human T-synthase. Cosmc, along with other ER chaperones such as HSP70 (BiP)/HSP40 and protein disulfide isomerase (PDI), assists its folding properly. Native T-synthase, which occurs mainly as a homodimer, exits to the Golgi apparatus, where it functions in synthesizing core 1 O-glycans (T-antigen). When Cosmc is mutated and dysfunctional, the nascent polypeptides of T-synthase form inactive aggregates or oligomers, which are associated with GRP78 (BiP), and subsequently proceed to the ERAD pathway where they are retrotranslocated from the ER to the cytosol, ubiquitinated, and subsequently degraded by the proteasome.