CSS3 is a glycosyltransferase that has both glucuronyltransferase and N-acetylgalactosaminyltransferase activities (Yada et al., 2003 [PubMed 12907687]).[supplied by OMIM, Mar 2008
Chondroitin sulfate synthases (CHSYs) synthesize chondroitin sulfate, a glycosaminoglycan expressed on the surface of most cells and in extracellular matrices. Glycosaminoglycan chains are covalently linked to various of core protein families and regulate many biologic processes, including extracellular matrix deposition, cell proliferation and recognition, and morphogenesis. The CHSY family includes CHSY1, CHSY2 and CHSY3. CHSY1 and CHSY3 display both glucuronyltransferase and N-acetylgalactosaminyltransferase activities, while CHSY2 is required for chondroitin polymerizing activity. CHSY2 localizes to the Golgi apparatus and is expressed ubiquitously, with highest expression observed in the pancreas, ovary, brain, heart, skeletal muscle, colon, kidney, liver, stomach, small intestine and placenta.
Function:
Has both beta-1,3-glucuronic acid and beta-1,4-N-acetylgalactosamine transferase activity. Transfers glucuronic acid (GlcUA) from UDP-GlcUA and N-acetylgalactosamine (GalNAc) from UDP-GalNAc to the non-reducing end of the elongating chondroitin polymer. Specific activity is much reduced compared to CHSY1.
Subcellular Location:
Golgi apparatus; Golgi stack membrane.
Tissue Specificity:
Detected at low levels in brain, cerebral cortex, uterus and small intestine.
Similarity:
Belongs to the chondroitin N-acetylgalactosaminyltransferase family.
SWISS:
Q70JA7
Gene ID:
337876
Database links:
Entrez Gene: 337876 Human
Omim: 609963 Human
SwissProt: Q70JA7 Human
Unigene: 213137 Human
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