Loading the page.
Proteins/UNIPROT:P17900
Lipid-binding and transfer protein essential for lysosomal degradation of ganglioside GM2. Extracts single GM2 molecules from intralysosomal luminal vesicle (ILV) membranes and presents them in soluble form to beta-hexosaminidase A (HEXA) for cleavage of N-acetyl-D-galactosamine and conversion to GM3. Forms a stoichiometric GM2-GM2A complex that serves as the substrate for HEXA. Also stimulates the breakdown of glycolipid GA2 by HEXA (By similarity). Possesses dual function: membrane lipid mobilization/solubilization and lipid substrate presentation to hydrolases. The large binding pocket can accommodate several single-chain phospholipids and fatty acids (By similarity). Exhibits some calcium-independent phospholipase activity (By similarity). Participates in cholesterol transfer..
Function from UniProtKB (P17900), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
1 row
140 statements
140 statements · 4 reference databases · Subject: GM2A · Sources: Hetionet, Human-GEM, Reactome, WikiPathways
6 reactions
a ganglioside GM2 (d18:1(4E))+MAM02012lMAM02013l
Catalysed by GM2A
Reaction pageLeft to rightBalance undeterminablelysosome
Human-GEM MAR00835CC BY 4.0Reference data
water+a ganglioside GM1 (d18:1(4E))alpha-D-galactose+a ganglioside GM2 (d18:1(4E))
5 names
Resolves to: GM2A
Reaction pageLeft to rightBalance undeterminablelysosome
Human-GEM MAR12036CC BY 4.0Reference data
MAM01904l+watera ganglioside GA2 (d18:1(4E))+alpha-D-galactose
Catalysed by CTSA or GALNS or GLB1 or GM2A or PSAP or NEU1
Reaction pageLeft to rightBalance undeterminablelysosome
Human-GEM MAR12037CC BY 4.0Reference data
a ganglioside GM2 (d18:1(4E))+watera ganglioside GM3 (d18:1(4E))+MAM02525l
Catalysed by HEXA & HEXB & GM2A
Reaction pageLeft to rightBalance undeterminableEC 3.2.1.52lysosome
Human-GEM MAR12061CC BY 4.0Reference data
a ganglioside GM2 (d18:1(4E))+watera ganglioside GM3 (d18:1(4E))+MAM02525e
Catalysed by HEXA & HEXB & GM2A
Reaction pageLeft to rightBalance undeterminableEC 3.2.1.52extracellular space
Human-GEM MAR12063CC BY 4.0Reference data
a ganglioside GA2 (d18:1(4E))+waterMAM02525l+MAM03586l
Catalysed by HEXA & HEXB & GM2A or HEXB & GM2A
Reaction pageLeft to rightBalance undeterminableEC 3.2.1.52lysosome
Human-GEM MAR12064CC BY 4.0Reference data