Metabolism Antibodies 2
Anti-GM2A Antibody (CAB6605)
- SKU:
- CAB6605
- Product Type:
- Antibody
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Description
| Antibody Name: | Anti-GM2A Antibody |
| Antibody SKU: | CAB6605 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB |
| Reactivity: | Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 24-193 of human GM2A (NP_000396.2). |
| Application: | WB |
| Recommended Dilution: | WB 1:500 - 1:2000 |
| Reactivity: | Mouse, Rat |
| Positive Samples: | Mouse liver, Rat liver |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 24-193 of human GM2A (NP_000396.2). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | HLKK PSQL SSFS WDNC DEGK DPAV IRSL TLEP DPII VPGN VTLS VMGS TSVP LSSP LKVD LVLE KEVA GLWI KIPC TDYI GSCT FEHF CDVL DMLI PTGE PCPE PLRT YGLP CHCP FKEG TYSL PKSE FVVP DLEL PSWL TTGN YRIE SVLS SSGK RLGC IKIA ASLK GI |
| Gene ID: | 2760 |
| Uniprot: | P17900 |
| Cellular Location: | Lysosome |
| Calculated MW: | 20kDa |
| Observed MW: | 26kDa |
| Synonyms: | GM2A, GM2-AP, SAP-3 |
| Background: | 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. |
| UniProt Protein Function: | GM2A: 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. Defects in GM2A are the cause of GM2-gangliosidosis type AB (GM2GAB); 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. |
| UniProt Protein Details: | Protein type:Activator; Mitochondrial Chromosomal Location of Human Ortholog: 5q33.1 Cellular Component: extracellular region; lysosomal lumen Molecular Function:sphingolipid activator protein activity Biological Process: glycosphingolipid metabolic process; neutrophil degranulation Disease: Gm2-gangliosidosis, Ab Variant |
| NCBI Summary: | 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] |
| UniProt Code: | P17900 |
| NCBI GenInfo Identifier: | 160331912 |
| NCBI Gene ID: | 2760 |
| NCBI Accession: | P17900.4 |
| UniProt Secondary Accession: | P17900,Q14426, Q14428, Q6LBL5, B2R699, D3DQH6, |
| UniProt Related Accession: | P17900 |
| Molecular Weight: | 21kDa |
| NCBI Full Name: | Ganglioside GM2 activator |
| NCBI Synonym Full Names: | GM2 ganglioside activator |
| NCBI Official Symbol: | GM2A |
| NCBI Official Synonym Symbols: | SAP-3; GM2-AP |
| NCBI Protein Information: | ganglioside GM2 activator |
| UniProt Protein Name: | Ganglioside GM2 activator |
| UniProt Synonym Protein Names: | Cerebroside sulfate activator protein; GM2-AP; Sphingolipid activator protein 3; SAP-3 |
| Protein Family: | Ganglioside GM2 activator |
| UniProt Gene Name: | GM2A |
 | Western blot analysis of extracts of various cell lines, using GM2A antibody (CAB6605) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Enhanced Kit (RM00021). Exposure time: 5min. |
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