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

General

glycoprotein membrane protein sulfotransferase

Properties

SIZE: entity length = 376 aa MW = 43 kD COMPARTMENT: golgi MOTIF: transmembrane domain {40-60} N-glycosylation site {N110} N-glycosylation site {N368}

Database Correlations

OMIM correlations MORBIDMAP 608429 UniProt Q8NCH0 Pfam PF03567 Entrez Gene 113189 Kegg hsa:113189 ENZYME 2.8.2.35

References

UniProt :accession Q8NCH0