Reactome | HS2ST1 trimer sulfates IdoA at C2 in heparan sulfate

HS2ST1 trimer sulfates IdoA at C2 in heparan sulfate

Stable Identifier

R-HSA-2076508

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi membrane, Golgi lumen

ReviewStatus

5/5

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HS2ST1 trimer sulfates IdoA at C2 in heparan sulfate

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Human heparan sulfate L-iduronyl 2-O-sulfotransferase 1 (HS2ST1) trimer mediates the transfer of sulfate from PAPS to the C2-position of iduronate (and glucuronate with lesser preference) (Smeds et al. 2010) residues in heparan sulfate (HS) chains of HS proteoglycans (Rong et al. 2000). Mutations in HS2ST1 can cause neurofacioskeletal syndrome (NFSRA, MIM:619194; Schneeberger et al., 2020).

Literature References

PubMed ID	Title	Journal	Year
10677367	Expression of heparan sulphate L-iduronyl 2-O-sulphotransferase in human kidney 293 cells results in increased D-glucuronyl 2-O-sulphation Rong, J, Habuchi, H, Kimata, K, Lindahl, U, Kusche-Gullberg, M	Biochem J	2000
20554947	Target selection of heparan sulfate hexuronic acid 2-O-sulfotransferase Smeds, E, Feta, A, Kusche-Gullberg, M	Glycobiology	2010
33159882	Bi-allelic Pathogenic Variants in HS2ST1 Cause a Syndrome Characterized by Developmental Delay and Corpus Callosum, Skeletal, and Renal Abnormalities Schneeberger, PE, von Elsner, L, Barker, EL, Meinecke, P, Marquardt, I, Alawi, M, Steindl, K, Joset, P, Rauch, A, Zwijnenburg, PJG, Weiss, MM, Merry, CLR, Kutsche, K	Am J Hum Genet	2020