| First Author | Wang Y | Year | 2010 |
| Journal | Proc Natl Acad Sci U S A | Volume | 107 |
| Issue | 20 | Pages | 9228-33 |
| PubMed ID | 20439703 | Mgi Jnum | J:160290 |
| Mgi Id | MGI:4454199 | Doi | 10.1073/pnas.0914004107 |
| Citation | Wang Y, et al. (2010) Cosmc is an essential chaperone for correct protein O-glycosylation. Proc Natl Acad Sci U S A 107(20):9228-33 |
| abstractText | Cosmc is a molecular chaperone thought to be required for expression of active T-synthase, the only enzyme that galactosylates the Tn antigen (GalNAcalpha1-Ser/Thr-R) to form core 1 Galbeta1-3GalNAcalpha1-Ser/Thr (T antigen) during mucin type O-glycan biosynthesis. Here we show that ablation of the X-linked Cosmc gene in mice causes embryonic lethality and Tn antigen expression. Loss of Cosmc is associated with loss of T-synthase but not other enzymes required for glycoprotein biosynthesis, demonstrating that Cosmc is specific in vivo for the T-synthase. We generated genetically mosaic mice with a targeted Cosmc deletion and survivors exhibited abnormalities correlated with Tn antigen expression that are related to several human diseases. |
