C1GALT1-specific chaperone 1 (core 1 beta3-galactosyltransferase- specific molecular chaperone, beta1,3-galactosyltransferase 2, C1GalT2, C1Gal-T2, C38H2-like protein 1, C38H2-L1, C1GALT1C1, COSMC, HSPC067, MSTP143, UNQ273/PRO310)
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Function
- probable chaperone, no transferase activity
- required for generation of 1 O-glycan Gal-beta1-3GalNAc-alpha1-Ser/Thr (T antigen), a precursor for many extended O-glycans in glycoproteins
- probably acts as a specific molecular chaperone assisting folding/stability of core 1 beta-3-galactosyltransferase (C1GALT1)
- associates with core 1 beta-3-galactosyltransferase (C1GALT1), probably not with the soluble active form
Structure
- belongs to the glycosyltransferase 31 family, Beta3- Gal-T subfamily
Compartment
membrane
Expression
- ubiquitously expressed
- expressed in salivary gland, stomach, small intestine, kidney, & testis > whole brain, cerebellum, spinal cord, thymus, spleen, trachea, lung, pancreas, ovary, & uterus
Pathology
- defects in C1GALT1C1 are the cause of Tn syndrome
- defects in C1GALT1C1 in Ag104A cell line create a tumor-specific glycopeptidic neo-epitope