CHST1

Description

The CHST1 (carbohydrate sulfotransferase 1) is a protein-coding gene located on chromosome 11.

Carbohydrate sulfotransferase 1 is an enzyme that in humans is encoded by the CHST1 gene.

CHST1 utilizes 3'-phospho-5'-adenylyl sulfate (PAPS) as a sulfonate donor to catalyze the transfer of sulfate to position 6 of internal galactose (Gal) residues within keratan sulfate. It collaborates with B4GALT4, B3GNT7 glycosyltransferases, and CHST6 sulfotransferase to construct and extend disulfated disaccharide units within the keratan sulfate polymer. CHST1 shows a preference for sulfating keratan sulfate but can also transfer sulfate to unsulfated polymers. It plays a role in the biosynthesis of phosphacan, a major keratan sulfate proteoglycan in brain development. Additionally, CHST1 is involved in the biosynthesis of 6-sulfoGalbeta-containing O-linked glycans in high endothelial venules of lymph nodes. It may work synergistically with CHST4 to produce sialyl 6',6-disulfo Lewis X motif, a recognition determinant for immune cell receptors involved in leukocyte trafficking. CHST1 efficiently catalyzes sulfation of N-acetyllactosamine (LacNAc) oligosaccharides, particularly sialylated LacNAc structures.

CHST1 is also known as C6ST, GST-1, KS6ST, KSGal6ST, KSST.

Associated Diseases

• type 2 diabetes mellitus
• neutropenia, severe congenital, 2, autosomal dominant
• combined immunodeficiency with skin granulomas
• common variable immunodeficiency
• immunodeficiency 18
• severe combined immunodeficiency due to IKK2 deficiency