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Carbohydrate sulfotransferase 1 (CHST1), also referred to as KSGal6ST or KSST, is a keratan sulfate sulfotransferase. It is predominantly expressed in brain and skeletal muscle and localizes to the trans-Golgi network. CHST1 is responsible for mediating the sulfation of keratan in the cornea, which is important in maintaining corneal transparency. In particular, CHST1 catalyzes the transfer of sulfate groups from 3'-phosphoadenosine 5'-phosphosulfate to position six of internal or terminal galactose residues (preferentially the residues adjacent to sulfated GlcNAc) on growing keratan sulfate chains. CHST1 also contributes to the generation of L-selectin ligands. Mutations in the CHST1 gene may play a role in macular corneal dystrophy (MCD).
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