Names & Taxonomy

Uniprot ID:
P17900
Entry Name:
SAP3_HUMAN
Status:
reviewed
Protein Names:
Ganglioside GM2 activator (Cerebroside sulfate activator protein) (GM2-AP) (Sphingolipid activator protein 3) (SAP-3) [Cleaved into: Ganglioside GM2 activator isoform short]
Gene Names:
GM2A
Gene Names Primary:
GM2A
Organism:
Homo sapiens (Human)

Structure

Length:
193
Sequence:
MQSLMQAPLLIALGLLLAAPAQAHLKKPSQLSSFSWDNCDEGKDPAVIRSLTLEPDPIIVPGNVTLSVMGSTSVPLSSPLKVDLVLEKEVAGLWIKIPCTDYIGSCTFEHFCDVLDMLIPTGEPCPEPLRTYGLPCHCPFKEGTYSLPKSEFVVPDLELPSWLTTGNYRIESVLSSSGKRLGCIKIAASLKGI
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Lysosome.

Function

Function:
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.
Cross Reference Drug Bank:
DB02325
Gene Ontology Go:
apical cortex
cytoplasmic side of plasma membrane
extracellular exosome
hydrogen:potassium-exchanging ATPase complex
lysosomal lumen
mitochondrion
beta-N-acetylgalactosaminidase activity
lipid transporter activity
phospholipase activator activity
ganglioside catabolic process
glycosphingolipid metabolic process
learning or memory
lipid storage
neuromuscular process controlling balance
oligosaccharide catabolic process
positive regulation of hydrolase activity
small molecule metabolic process
sphingolipid metabolic process
Gene Ontology Biological Process:
ganglioside catabolic process
glycosphingolipid metabolic process
learning or memory
lipid storage
neuromuscular process controlling balance
oligosaccharide catabolic process
positive regulation of hydrolase activity
small molecule metabolic process
sphingolipid metabolic process
Gene Ontology Molecular Function:
beta-N-acetylgalactosaminidase activity
lipid transporter activity
phospholipase activator activity
Gene Ontology Cellular Component:
apical cortex
cytoplasmic side of plasma membrane
extracellular exosome
hydrogen:potassium-exchanging ATPase complex
lysosomal lumen
mitochondrion
Keywords:
3D-structure
Complete proteome
Direct protein sequencing
Disease mutation
Disulfide bond
Gangliosidosis
Glycoprotein
Hydrolase
Lipid metabolism
Lysosome
Polymorphism
Reference proteome
Signal
Sphingolipid metabolism

Publication

PubMed ID:
2059210 1915857 1554364 1427911 14702039 15372022 10364519 15489334 2753159 2209618 25944712 11090283 1915858 8244332 8900233