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GM2 Ganglioside Activator (GM2A)

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SAP-3; Cerebroside Sulfate Activator Protein; Sphingolipid Activator Protein 3

GM2 Ganglioside Activator (GM2A)
CCC L122
HGNC 4367
MGI 95762
PubMed 11339652
RGD 628651
UniProt P17900
Wiki GM2A
GM2A is a small glycolipid transport protein which acts as a substrate specific co-factor for the lysosomal enzyme β-hexosaminidase A. β-hexosaminidase A, together with GM2 ganglioside activator, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines.
GM2A is a lipid transfer protein that stimulates the enzymatic processing of gangliosides, and also T-cell activation through lipid presentation. This protein binds molecules of ganglioside GM2, extracts them from membranes, and presents them to beta-hexosaminidase A for cleavage of N-acetyl-D-galactosamine and conversion to GM3.It was identified as a member of ML domain family of proteins involved in innate immunity and lipid metabolism in the SMART database.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPL122Hu01 Recombinant GM2 Ganglioside Activator (GM2A) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAL122Hu01 Polyclonal Antibody to GM2 Ganglioside Activator (GM2A) WB; IHC; ICC; IP.
MAL122Hu21 Monoclonal Antibody to GM2 Ganglioside Activator (GM2A) WB; IHC; ICC; IP.
Assay Kits SEL122Hu ELISA Kit for GM2 Ganglioside Activator (GM2A) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant GM2 Ganglioside Activator (GM2A) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to GM2 Ganglioside Activator (GM2A) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to GM2 Ganglioside Activator (GM2A) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for GM2 Ganglioside Activator (GM2A) CLIA Kit Customized Service Offer
n/a ELISA Kit for GM2 Ganglioside Activator (GM2A) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant GM2 Ganglioside Activator (GM2A) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to GM2 Ganglioside Activator (GM2A) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to GM2 Ganglioside Activator (GM2A) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for GM2 Ganglioside Activator (GM2A) CLIA Kit Customized Service Offer
n/a ELISA Kit for GM2 Ganglioside Activator (GM2A) ELISA Kit Customized Service Offer
  1. "Isolation and expression of a full-length cDNA encoding the human G-M2 activator protein."Biochem. Biophys. Res. Commun. 177:1217-1223(1991) [PubMed] [Europe PMC] [Abstract]
  2. "Characterization of full-length cDNAs and the gene coding for the human GM2 activator protein."FEBS Lett. 289:260-264(1991) [PubMed] [Europe PMC] [Abstract]
  3. "Evidence for two cDNAs encoding human GM2-activator protein."Biochem. J. 282:807-813(1992) [PubMed] [Europe PMC] [Abstract]
  4. "Identification of a processed pseudogene related to the functional gene encoding the GM2 activator protein: localization of the pseudogene to human chromosome 3 and the functional gene to human chromosome 5."Genomics 14:796-798(1992) [PubMed] [Europe PMC] [Abstract]
  5. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  6. "The DNA sequence and comparative analysis of human chromosome 5." Nature 431:268-274(2004) [PubMed] [Europe PMC] [Abstract]
  7. "Structure of the GM2A gene: identification of an exon 2 nonsense mutation and a naturally occurring transcript with an in-frame deletion of exon 2."Am. J. Hum. Genet. 65:77-87(1999) [PubMed] [Europe PMC] [Abstract]
  8. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  9. "Isolation of a cDNA encoding the human GM2 activator protein."FEBS Lett. 251:197-200(1989) [PubMed] [Europe PMC] [Abstract]
  10. "The complete amino-acid sequences of human ganglioside GM2 activator protein and cerebroside sulfate activator protein."Eur. J. Biochem. 192:709-714(1990) [PubMed] [Europe PMC] [Abstract]
  11. "Crystal structure of human GM2-activator protein with a novel beta-cup topology."J. Mol. Biol. 304:411-422(2000) [PubMed] [Europe PMC] [Abstract]
  12. "A mutation in the gene of a glycolipid-binding protein (GM2 activator) that causes GM2-gangliosidosis variant AB."FEBS Lett. 290:1-3(1991) [PubMed] [Europe PMC] [Abstract]
  13. "Molecular genetics of GM2-gangliosidosis AB variant: a novel mutation and expression in BHK cells."Hum. Genet. 92:437-440(1993) [PubMed] [Europe PMC] [Abstract]
  14. "Molecular analysis of a GM2-activator deficiency in two patients with GM2-gangliosidosis AB variant."Am. J. Hum. Genet. 59:1048-1056(1996) [PubMed] [Europe PMC] [Abstract]