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Product Name GM2A Chinese Name 神经鞘脂激活蛋白3抗体 Alias Cerebroside sulfate activator protein; Ganglioside GM2 activator isoform short; Ganglioside GM2 activator precursor; 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. Research Area Tumour Cell biology Neurobiology Cell type markers The new supersedes the old Immunogen Species Rabbit Clonality Polyclonal React Species Human, Rat, Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 17/18kDa Cellular localization cytoplasmic Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human GM2A/SAP3: 131-193/193 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail This gene encodes a small glycolipid transport protein which acts as a substrate specific co-factor for the lysosomal enzyme beta-hexosaminidase A. Beta-hexosaminidase A, together with GM2 ganglioside activator, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene result in GM2-gangliosidosis type AB or the AB variant of Tay-Sachs disease. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Nov 2009].
Function:
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.
Subcellular Location:
Lysosome.
Post-translational modifications:
The serines in positions 32 and 33 are absent in 80% of the sequenced protein.
DISEASE:
Defects in GM2A are the cause of GM2-gangliosidosis type AB (GM2GAB) [MIM:272750]; also known as Tay-Sachs disease AB variant. GM2-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2GAB is characterized by GM2 gangliosides accumulation in the presence of both hexosaminidase A and B.
SWISS:
P17900
Gene ID:
2760
Database links:Entrez Gene: 2760 Human
Entrez Gene: 14667 Mouse
Omim: 613109 Human
SwissProt: P17900 Human
SwissProt: Q60648 Mouse
Unigene: 483873 Human
Unigene: 287807 Mouse
Unigene: 98783 Rat
Product Picture Tissue/cell: human kidney tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-GM2A Polyclonal Antibody, Unconjugated(SL13452R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
Tissue/cell: Rat testis tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-GM2A Polyclonal Antibody, Unconjugated(SL13452R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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