carbohydrate sulfotransferase 14; dermatan 4-sulfotransferase 1; D4ST-1; hD4ST1 (CHST14, D4ST1, UNQ1925/PRO4400)

Function

• catalyzes transfer of sulfate to position 4 of the N-acetylgalactosamine (galNAc) residue of dermatan sulfate
• role in formation of 4-0-sulfated IdoA blocks in dermatan sulfate
• transfers sulfate to the C-4 hydroxyl of beta1,4-linked galNAc that is substituted with an alpha-linked iduronic acid (IdoUA) at the C-3 hydroxyl
• transfers sulfate more efficiently to galNAc residues in -IdoUA-galNAc-IdoUA- than in -GlcUA-galNAc-GlcUA-sequences
• preference for partially desulfated dermatan sulfate
• addition of sulfate to galNAc may occur immediately after epimerization of GlcUA to IdoUA
• role in formation of cerebellar neural network during postnatal brain development (putative)

3'-phospho-5'-adenylyl sulfate + [dermatan]-N-acetyl-D-galactosamine = adenosine 3',5'-bisphosphate + [dermatan]- 4-O-sulfo-N-acetyl-D-galactosamine

Structure

belongs to the sulfotransferase 2 family

Compartment

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

Expression

• widely expressed
• expressed at high level in pituitary gland, placenta, uterus & thyroid

Pathology

• defects in CHST14 are the cause of Ehlers-Danlos syndrome musculocontractural type

More general terms

• sulfotransferase
• glycoprotein
• membrane protein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=113189
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:113189
• OMIM: https://mirror.omim.org/entry/601776
• OMIM: https://mirror.omim.org/entry/608429
• UniProt: http://www.uniprot.org/uniprot/Q8NCH0.html