Reaction: Defective ARSB does not hydrolyse DS
- in pathway: MPS VI - Maroteaux-Lamy syndrome
Arylsulfatase B using calcium cofactor (ARSB:Ca2+) hydrolyses sulfate from N-acetylgalactosamine 4-sulfate (or 6-sulfate) units (GalNAc 4-sulfate or GalNAc 6-sulfate) within chondroitin sulfate (represented here by Chebi:63519 chain). Defects in ARSB cause mucopolysaccharidosis type VI (MPS VI, Maroteaux-Lamy syndrome, polydystrophic dwarfism; MIM:253200), an autosomal recessive lysosomal storage disorder. Severe forms of the disease are caused by the ARSB mutations Y86del (Karageorgos et al. 2004), P116H (Villani et al. 1999), C117R (Jin et al. 1992), G144R (Isbrandt et al. 1994) and R95Q/H393P (Litjens et al. 1996).
Reaction - small molecule participants:
H2O [lysosomal lumen]
ChEBI:63519 chain [lysosomal lumen]
Reactome.org reaction link: R-HSA-9036065
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Reaction input - small molecules:
water
beta-D-GalNAc4S-(1->4)-beta-D-IdoA2S-(1->3)-beta-D-GalNAc4S-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl-yl group
Reaction output - small molecules:
Reactome.org link: R-HSA-9036065