Reactome | HS6STs sulfate GlcN at C6 in heparan sulfate/heparin

HS6STs sulfate GlcN at C6 in heparan sulfate/heparin

Stable Identifier

R-HSA-2076419

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi membrane, Golgi lumen

ReviewStatus

5/5

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HS6STs sulfate GlcN at C6 in heparan sulfate/heparin

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Heparan-sulfate 6-O-sulfotransferases 1 and 2 (HS3ST1-2) (Habuchi et al. 1998, Habuchi et al. 2003 respectively) catalyze the transfer of sulfate to C6 of the N-sulfoglucosamine residue (GlcNS) of heparan sulfate. A third member HS3ST3 that may also mediate this reaction has been characterised in mouse (Habuchi et al. 2000) but remains uncharacterised in humans. It can be argued that this structure can now be called either heparan sulfate- or heparin-PG. Mutations in HS3ST1 can cause hypogonadotropic hypogonadism (HH13, MIM:614880; Tornberg et al., 2011). Mutations in HS6ST2 can cause the neurodevelopmental disorder named Paganini-Miozzo syndrome (MRXSPM, MIM:301025; Paganini et al., 2019).

Literature References

PubMed ID	Title	Journal	Year
10644753	The occurrence of three isoforms of heparan sulfate 6-O-sulfotransferase having different specificities for hexuronic acid adjacent to the targeted N-sulfoglucosamine Habuchi, H, Tanaka, M, Habuchi, O, Yoshida, K, Suzuki, H, Ban, K, Kimata, K	J Biol Chem	2000
12492399	Biosynthesis of heparan sulphate with diverse structures and functions: two alternatively spliced forms of human heparan sulphate 6-O-sulphotransferase-2 having different expression patterns and properties Habuchi, H, Miyake, G, Nogami, K, Kuroiwa, A, Matsuda, Y, Kusche-Gullberg, M, Habuchi, O, Tanaka, M, Kimata, K	Biochem J	2003
21700882	Heparan sulfate 6-O-sulfotransferase 1, a gene involved in extracellular sugar modifications, is mutated in patients with idiopathic hypogonadotrophic hypogonadism Tornberg, J, Sykiotis, GP, Keefe, K, Plummer, L, Hoang, X, Hall, JE, Quinton, R, Seminara, SB, Hughes, V, Van Vliet, G, Van Uum, S, Crowley, WF, Habuchi, H, Kimata, K, Pitteloud, N, Bülow, HE	Proc Natl Acad Sci U S A	2011
30471091	A HS6ST2 gene variant associated with X-linked intellectual disability and severe myopia in two male twins Paganini, L, Hadi, LA, Chetta, M, Rovina, D, Fontana, L, Colapietro, P, Bonaparte, E, Pezzani, L, Marchisio, P, Tabano, SM, Costanza, J, Sirchia, SM, Riboni, L, Milani, D, Miozzo, M	Clin Genet	2019
9535912	Molecular characterization and expression of heparan-sulfate 6-sulfotransferase. Complete cDNA cloning in human and partial cloning in Chinese hamster ovary cells Habuchi, H, Kobayashi, M, Kimata, K	J Biol Chem	1998