Recombinant Human Ganglioside GM2 activator (GM2A)

Code CSB-EP009565HUe0
Size $224
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  • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.

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Product Details

Purity
Greater than 85% as determined by SDS-PAGE.
Target Names
GM2A
Uniprot No.
Research Area
Signal Transduction
Alternative Names
Cerebroside sulfate activator protein; ganglioside GM2 activator; Ganglioside GM2 activator isoform short; Ganglioside GM2 activator precursor; GM2 activator; GM2 AP; GM2 ganglioside activator; GM2 ganglioside activator protein; GM2-AP; GM2A; GM2AP; OTTHUMP00000160619; SAP 3; SAP-3; SAP3; SAP3_HUMAN; Shingolipid activator protein 3; Sphingolipid activator protein 3
Species
Homo sapiens (Human)
Source
E.coli
Expression Region
32-193aa
Target Protein Sequence
SSFSWDNCDEGKDPAVIRSLTLEPDPIIVPGNVTLSVMGSTSVPLSSPLKVDLVLEKEVAGLWIKIPCTDYIGSCTFEHFCDVLDMLIPTGEPCPEPLRTYGLPCHCPFKEGTYSLPKSEFVVPDLELPSWLTTGNYRIESVLSSSGKRLGCIKIAASLKGI
Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
Mol. Weight
44.6 kDa
Protein Length
Full Length of Mature Protein
Tag Info
N-terminal GST-tagged
Form
Liquid or Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
Buffer
If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol.
Note: If you have any special requirement for the glycerol content, please remark when you place the order.
If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.
Reconstitution
We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20°C/-80°C. Our default final concentration of glycerol is 50%. Customers could use it as reference.
Troubleshooting and FAQs
Storage Condition
Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life
The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Lead Time
Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Notes
Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet & COA
Please contact us to get it.

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Target Background

Function
The large binding pocket can accommodate several single chain phospholipids and fatty acids, GM2A also exhibits some calcium-independent phospholipase activity. 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. Has cholesterol transfer activity.
Gene References into Functions
  1. Review of GM2A mutations causing GM2 activator protein deficiency and GM2 gangliosidosis-AB variant. PMID: 27402091
  2. Studies indicate that sphingolipid activator proteins (SAPs) and anionic lipids are essential stimulators to reach physiological rates of lysosomal sphingolipid degradation. PMID: 27157270
  3. this study has established the potential role of GM2A in breast cancer progression PMID: 27002480
  4. Mobilization of membrane lipids by GM2AP was also inhibited in the presence of cholesterol or SM, as revealed by surface plasmon resonance studies PMID: 26175473
  5. Gene polymorphisms of MD2 and GM2A were associated with the occurrence or severity of neonatal necrotizing enterocolitis. PMID: 25816011
  6. In vitro assays with the isolated H1 or H2 homodimers (beta-alpha hybrid construct of beta-hexosaminidase A subunits) confirmed that neither was capable of human GM2AP-dependent hydrolysis of GM2 ganglioside. PMID: 23483939
  7. impact of GM2AP on glucose metabolism PMID: 21784073
  8. Treatment of meniscal explants with IL-1RA inhibited the expression of many catabolic genes following a single bout of high dynamic strain. PMID: 21331553
  9. these results provide novel insights into the physiological functions of GM2AP in obesity. PMID: 21036149
  10. GM2 activator protein exerts strong and broad inhibitory effects on the hydrolysis of phospholipids carried out by plant and microbial phospholipases D PMID: 12576516
  11. Two new structures of GM2-AP with bound lipids, showing two different lipid-binding modes within the apolar pocket PMID: 12909021
  12. elucidation of mode of action on gangioside GM2 PMID: 14728689
  13. alpha-subunit loop structure is required for GM2 activator protein binding by beta-hexosaminidase A PMID: 15485660
  14. glycosphingolipids, particularly GM2, form a complex with CD82, and this complex interacts with Met and thereby inhibits HGF-induced Met tyrosine kinase activity, as well as integrin to Met cross-talk PMID: 17215249

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Involvement in disease
GM2-gangliosidosis AB (GM2GAB)
Subcellular Location
Lysosome.
Database Links

HGNC: 4367

OMIM: 272750

KEGG: hsa:2760

STRING: 9606.ENSP00000349687

UniGene: Hs.483873

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