carbohydrate sulfotransferase 6; corneal N-acetylglucosamine-6-O-sulfotransferase; C-GlcNAc6ST; hCGn6ST; galactose/N-acetylglucosamine/N-acetylglucosamine 6-O-sulfotransferase 4-beta; GST4-beta; N-acetylglucosamine 6-O-sulfotransferase 5; GlcNAc6ST-5; Gn6st-5 (CHST6)

Function

• catalyzes transfer of sulfate to position 6 of non-reducing N-acetylglucosamine (GlcNAc) residues of keratan
• mediates sulfation of keratan in cornea
• keratan sulfate plays a central role in maintaining corneal transparency
• acts on the non-reducing terminal GlcNAc of short & long carbohydrate substrates that have poly-N-acetyllactosamine structures

Structure

• belongs to the sulfotransferase 1 family gal/GlcNAc/galNAc subfamily

Compartment

• Golgi apparatus membrane
• single-pass type 2 membrane protein (putative)

Expression

• expressed in cornea
• mainly expressed in brain
• also expressed in spinal cord & trachea

Pathology

• defects in CHST6 are the cause of macular corneal dystrophy

More general terms

• carbohydrate sulfotransferase (CHST)

Additional terms

• macular corneal dystrophy

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=4166
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:4166
• OMIM: https://mirror.omim.org/entry/217800
• OMIM: https://mirror.omim.org/entry/605294
• UniProt: http://www.uniprot.org/uniprot/Q9GZX3.html