Beta-galactosidase hydrolyses GM1 to GM2

Stable Identifier
Reaction [transition]
Homo sapiens
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Gangliosides are glycosphingolipids in which oligosaccharide chains containing N-acetylneuraminic acid (NeuNAc) are attached to a ceramide. The prototypical ganglioside GM1 can be hydrolysed to the GM2 ganglioside by beta-galactosidase (GLB1), cleaving off the terminal galactose (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
1928092 Human beta-galactosidase gene mutations in morquio B disease

Oshima, A, Yoshida, K, Shimmoto, M, Fukuhara, Y, Sakuraba, H, Suzuki, Y

Am J Hum Genet 1991
5822067 Human small-intestinal beta-galactosidases. Separation and characterization of one lactase and one hetero beta-galactosidase

Asp, NG, Dahlqvist, A, KoldovskĆ½, O

Biochem J 1969
1907800 Human beta-galactosidase gene mutations in GM1-gangliosidosis: a common mutation among Japanese adult/chronic cases

Yoshida, K, Oshima, A, Shimmoto, M, Fukuhara, Y, Sakuraba, H, Yanagisawa, N, Suzuki, Y

Am J Hum Genet 1991
Catalyst Activity

beta-galactosidase activity of BGAL [lysosomal lumen]

Orthologous Events
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