Reactome | GUSB tetramer hydrolyses CS/HS precursor

GUSB tetramer hydrolyses CS/HS precursor

Stable Identifier

R-HSA-1678854

Type

Reaction [transition]

Species

Homo sapiens

Compartment

lysosomal lumen

ReviewStatus

5/5

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GUSB tetramer hydrolyses CS/HS precursor

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The tetrameric lysosomal enzyme beta-glucuronidase (GUSB) hydrolyses glucuronate from heparan or the linker chain (Oshima et al. 1987). L-aspartic acid is an inhibitor of enzyme activity (Kreamer et al. 2001). Mutations in GUSB can cause Mucopolysaccharidosis 7 (MPS7, aka Sly syndrome, MIM:253220; reviewed in Tomatsu et al., 2009).

Literature References

PubMed ID	Title	Journal	Year
19224584	Mutations and polymorphisms in GUSB gene in mucopolysaccharidosis VII (Sly Syndrome) Tomatsu, S, Montaño, AM, Dung, VC, Grubb, JH, Sly, WS	Hum Mutat	2009
3468507	Cloning, sequencing, and expression of cDNA for human beta-glucuronidase Oshima, A, Kyle, JW, Miller, RD, Hoffmann, JW, Powell, PP, Grubb, JH, Sly, WS, Tropak, M, Guise, KS, Gravel, RA	Proc Natl Acad Sci U S A	1987
11568288	A novel inhibitor of beta-glucuronidase: L-aspartic acid Kreamer, BL, Siegel, FL, Gourley, GR	Pediatr Res	2001