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CHST6 Sulfotransferase that utilizes 3'-phospho-5'-adenylyl sulfate (PAPS) as sulfonate donor to catalyze the 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 and long carbohydrate substrates that have poly-N- acetyllactosamine structures. Defects in CHST6 are the cause of macular corneal dystrophy (MCD). MCD is an autosomal recessive disease characterized by corneal opacities. Onset occurs in the first decade, usually between ages 5 and 9. The disorder is progressive. Minute, gray, punctate opacities develop. Corneal sensitivity is usually reduced. Painful attacks with photophobia, foreign body sensations, and recurrent erosions occur in most patients. There are different types of MCD: MCD type I, in which there is a virtual absence of sulfated keratan sulfate (KS) in the serum and cornea, as determined by KS-specific antibodies; and MCD type II, in which the normal sulfated KS-antibody response is present in cornea and serum. MCD type I patients usually have a homozygous missense mutation, while MCD type II patients show a large deletion and replacement in the upstream region of CHST6. The only missense mutation for type II is Cys-50, which is heterozygous with a replacement in the upstream region on the other allele of CHST6. Belongs to the sulfotransferase 1 family. Gal/GlcNAc/GalNAc subfamily. Note: This description may include information from UniProtKB.
Protein type: EC 2.8.2.-; Glycan Metabolism - keratan sulfate biosynthesis; Membrane protein, integral; Transferase
Chromosomal Location of Human Ortholog: 16q23.1
Cellular Component: Golgi apparatus; Golgi membrane; integral component of membrane
Molecular Function: N-acetylglucosamine 6-O-sulfotransferase activity
Biological Process: carbohydrate metabolic process; keratan sulfate biosynthetic process; N-acetylglucosamine metabolic process; sulfur compound metabolic process
Disease: Macular Dystrophy, Corneal, 1
Reference #:  Q9GZX3 (UniProtKB)
Alt. Names/Synonyms: C-GlcNAc6ST; carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 6; Carbohydrate sulfotransferase 6; CHST6; corneal N-acetylglucosamine 6-sulfotransferase; Corneal N-acetylglucosamine-6-O-sulfotransferase; Galactose/N-acetylglucosamine/N-acetylglucosamine 6-O-sulfotransferase 4-beta; GlcNAc6ST-5; Gn6st-5; GST4-beta; hCGn6ST; MCDC1; N-acetylglucosamine 6-O-sulfotransferase 5
Gene Symbols: CHST6
Molecular weight: 44,099 Da
Basal Isoelectric point: 10.03  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

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