UniProt Accession

 SAP3_HUMAN; P17900; B2R699; D3DQH6; Q14426; Q14428; Q6LBL5 

Genbank Protein ID

 M76477; X62078; X62078; X61095; L01439; AF124719; AF124717; AF124718; AK312494; AC008385; CH471062; CH471062; BC009273; X16087 

Genbank Nucleotide ID

 AAA35907.1; CAA43993.1; CAA43994.1; CAA43408.1; AAA52767.1; AAD25741.1; AAD25741.1; AAD25741.1; BAG35396.1; EAW61680.1; EAW61681.1; AAH09273.1; CAA34215.1 

Protein Name

 Ganglioside GM2 activator 

Protein Synonyms/Alias

 Cerebroside sulfate activator protein; GM2-AP; Sphingolipid activator protein 3; SAP-3; Ganglioside GM2 activator isoform short 

Gene Name

 GM2A 

Gene Synonyms/Alias

  

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
179	SVLSSSGKRLGCIKI	ubiquitination	[1]

Reference

 [1] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
 Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
 Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961] 

Functional Description

 The large binding pocket can accommodate several single chain phospholipids and fatty acids, GM2A also exhibits some calcium-independent phospholipase activity (By similarity). Binds gangliosides and stimulates ganglioside GM2 degradation. It stimulates only the breakdown of ganglioside GM2 and glycolipid GA2 by beta-hexosaminidase A. It extracts single GM2 molecules from membranes and presents them in soluble form to beta- hexosaminidase A for cleavage of N-acetyl-D-galactosamine and conversion to GM3. 

Sequence Annotation

 CARBOHYD 63 63 N-linked (GlcNAc...).
 DISULFID 39 183
 DISULFID 99 106
 DISULFID 112 138
 DISULFID 125 136  

Keyword

 3D-structure; Complete proteome; Direct protein sequencing; Disease mutation; Disulfide bond; Gangliosidosis; Glycoprotein; Hydrolase; Lipid metabolism; Lysosome; Polymorphism; Reference proteome; Signal; Sphingolipid metabolism. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 193 AA 

Protein Sequence

Gene Ontology

 GO:0043202; C:lysosomal lumen; TAS:Reactome.
 GO:0005739; C:mitochondrion; IEA:Compara.
 GO:0005730; C:nucleolus; IDA:HPA.
 GO:0004563; F:beta-N-acetylhexosaminidase activity; IEA:Compara.
 GO:0030290; F:sphingolipid activator protein activity; NAS:UniProtKB.
 GO:0006689; P:ganglioside catabolic process; IEA:Compara.
 GO:0019377; P:glycolipid catabolic process; NAS:UniProtKB.
 GO:0006687; P:glycosphingolipid metabolic process; TAS:Reactome.
 GO:0007611; P:learning or memory; IEA:Compara.
 GO:0019915; P:lipid storage; IEA:Compara.
 GO:0050885; P:neuromuscular process controlling balance; IEA:Compara.
 GO:0009313; P:oligosaccharide catabolic process; IEA:Compara.
 GO:0044281; P:small molecule metabolic process; TAS:Reactome.
 GO:0030149; P:sphingolipid catabolic process; NAS:UniProtKB. 

Interpro

 IPR003172; MD-2_lipid-recog_dom. 

Pfam

  

SMART

 SM00737; ML 

PROSITE

  

PRINTS