Reactome | GLB1 hydrolyses linker chain(2)

GLB1 hydrolyses linker chain(2)

Stable Identifier

R-HSA-2090079

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 the linker chain sequence of glycosaminoglycans (Asp et al. 1969). Defects in GLB1 causes the lysosomal storage diseases GM1 gangliosidosis (Yoshida et al. 1991) and Morquio syndrome 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