Gm2a
entrez
14667
canonical
uniprot
Q60648
canonical
ensembl
ENSMUSG00000000594
resolver
ensp
ENSMUSP00000000608
resolver
enst
ENSMUST00000000608
resolver
genesymbol
Gm2a
resolver
uniprot
Q5F1Z8
resolver
uniprot
Q61610
resolver
uniprot
Q61819
resolver
uniprot_entry
Q5F1Z8_MOUSE
resolver
uniprot_entry
SAP3_MOUSE
resolver
uniprot_sec
Q61610
resolver
uniprot_sec
Q61819
resolver
activity_regulation
ACTIVITY REGULATION: Activity is inhibited by cholesterol, sphingomyelin, sphingosine, and sphinganine. Activity is stimulated by ceramide, diacylglycerol, fatty acids, lysophosphatidylcholine, and anionic lipids such as BMP (bis(monoacylglycero)phosphate). {ECO:0000250|UniProtKB:P17900}.
uniprot
proteins
amino_acid_sequence
MHRLPLLLLLGLLLAGSVAPARLVPKRLSQLGGFSWDNCDEGKDPAVIKSLTIQPDPIVVPGDVVVSLEGKTSVPLTAPQKVELTVEKEVAGFWVKIPCVEQLGSCSYENICDLIDEYIPPGESCPEPLHTYGLPCHCPFKEGTYSLPTSNFTVPDLELPSWLSTGNYRIQSILSSGGKRLGCIKIAASLKGR
uniprot
proteins
function
FUNCTION: 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 (By similarity). Also stimulates the breakdown of glycolipid GA2 by HEXA (PubMed:16216074). Possesses dual function: membrane lipid mobilization/solubilization and lipid substrate presentation to hydrolases (By similarity). The large binding pocket can accommodate several single-chain phospholipids and fatty acids. Exhibits some calcium-independent phospholipase activity (PubMed:16216074). Participates in cholesterol transfer (By similarity). {ECO:0000250|UniProtKB:P17900, ECO:0000269|PubMed:16216074}.
uniprot
proteins
mass_dalton
20824
uniprot
proteins
mutagenesis
MUTAGEN 168; /note="Y->S: Abolishes phospholipid binding."; /evidence="ECO:0000269|PubMed:16216074
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16216074
uniprot
proteins
pubmed
21183079
uniprot
proteins
pubmed
7689829
uniprot
proteins
pubmed
7713516
uniprot
proteins
pubmed
9060405
uniprot
proteins
sequence_length
193
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Lysosome {ECO:0000250|UniProtKB:P17900}.
uniprot
proteins