CHST6
Description
The CHST6 (carbohydrate sulfotransferase 6) is a protein-coding gene located on chromosome 16.
CHST6 (Carbohydrate sulfotransferase 6) is an enzyme produced by the human CHST6 gene. This enzyme is essential for the production of keratan sulfate, a crucial component of the cornea. Mutations in the CHST6 gene lead to macular corneal dystrophy, a condition that affects corneal clarity.
CHST6 is a sulfotransferase that transfers sulfate from 3'-phospho-5'-adenylyl sulfate (PAPS) to the 6th position of non-reducing N-acetylglucosamine (GlcNAc) residues in keratan sulfate. It works together with B4GALT4 galactosyltransferase and B3GNT7 N-acetylglucosaminyltransferase to build and extend the sulfated disaccharide unit [->3Galbeta1->4(6-sulfoGlcNAcbeta)1->] within the keratan sulfate polymer. CHST6 is involved in keratan sulfate synthesis in the cornea, affecting proteoglycan fibril organization and corneal transparency. It also participates in the sulfation of endothelial mucins like GLYCAM1.
CHST6 is also known as C-GlcNAc6ST, GST4-beta, MCDC1, glcNAc6ST-5, gn6st-5, hCGn6ST.
