Reactome | GLB1 hydrolyses a glycosaminoglycan

GLB1 hydrolyses a glycosaminoglycan

Stable Identifier

R-HSA-1630306

Type

Reaction [transition]

Species

Homo sapiens

Compartment

lysosomal lumen

ReviewStatus

5/5

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Beta-galactosidase (GLB1) can cleave terminal galactose residues from glycosaminoglycans such as keratan sulfate (KS) (Asp et al. 1969). Defects in GLB1 cause the lysosomal storage diseases GM1gangliosidosis (Yoshida et al. 1991) and Morquio syndrome type B (Oshima et al. 1991).

Literature References

PubMed ID	Title	Journal	Year
5822067	Human small-intestinal beta-galactosidases. Separation and characterization of one lactase and one hetero beta-galactosidase Koldovský, O, Asp, NG, Dahlqvist, A	Biochem J	1969
1928092	Human beta-galactosidase gene mutations in morquio B disease Fukuhara, Y, Yoshida, K, Suzuki, Y, Sakuraba, H, Oshima, A, Shimmoto, M	Am J Hum Genet	1991
1907800	Human beta-galactosidase gene mutations in GM1-gangliosidosis: a common mutation among Japanese adult/chronic cases Fukuhara, Y, Yoshida, K, Suzuki, Y, Sakuraba, H, Oshima, A, Yanagisawa, N, Shimmoto, M	Am J Hum Genet	1991