GM2A binds and mobilizes ligands

Stable Identifier
R-HSA-1605717
Type
Reaction [transition]
Species
Homo sapiens
Compartment
ReviewStatus
5/5
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Ganglioside GM2 activator (GM2A, GM2AP) acts as carrier protein solubilizing the membrane-bound glycolipids GM1, GM2, GA1, GA2, and SM2A, functioning as a cofactor in their enzymatic degradation. GM2A is essential for the hydrolysis of GM2, and defects in GM2A function lead to GM2 gangliosidosis AB (MIM:272750), also known as Tay-Sachs disease AB variant (Schroeder et al., 1991; Wilkening et al., 2000; reviewed by Sandhoff & Sandhoff, 2018).

Literature References
PubMed ID Title Journal Year
29802621 Emerging concepts of ganglioside metabolism

Sandhoff, R, Sandhoff, K

FEBS Lett 2018
10942779 Degradation of membrane-bound ganglioside GM1. Stimulation by bis(monoacylglycero)phosphate and the activator proteins SAP-B and GM2-AP

Wilkening, G, Linke, T, Uhlhorn-Dierks, G, Sandhoff, K

J Biol Chem 2000
12909021 Structural analysis of lipid complexes of GM2-activator protein

Wright, CS, Rastinejad, F, Zhao, Q

J Mol Biol 2003
1915858 A mutation in the gene of a glycolipid-binding protein (GM2 activator) that causes GM2-gangliosidosis variant AB

Suzuki, K, Sandhoff, K, Schröder, M, Schnabel, D

FEBS Lett 1991
Participants
Participates
Catalyst Activity

sphingolipid activator protein activity of GM2A(32-193) [lysosomal lumen]

Orthologous Events
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