Mut Antibody (PACO49014)
- SKU:
- PACO49014
- Product Type:
- Antibody
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- ELISA
- Antibody Type:
- Polyclonal Antibody
- Conjugation:
- Unconjugated
Description
| Antibody Name: | Mut Antibody (PACO49014) |
| Antibody SKU: | PACO49014 |
| Size: | 50ug |
| Host Species: | Rabbit |
| Tested Applications: | ELISA |
| Recommended Dilutions: | |
| Species Reactivity: | Mouse |
| Immunogen: | Recombinant Mouse Methylmalonyl-CoA mutase, mitochondrial protein (31-748AA) |
| Form: | Liquid |
| Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
| Purification Method: | >95%, Protein G purified |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Non-conjugated |
| Background: | Involved in the degradation of several amino acid, , odd-chain fatty acid, and cholesterol via propionyl-CoA to the tricarboxylic acid, cycle. |
| Synonyms: | Methylmalonyl-CoA mutase, mitochondrial (MCM) (EC 5.4.99.2) (Methylmalonyl-CoA isomerase), Mut |
| UniProt Protein Function: | MUT: Involved in the degradation of several amino acids, odd- chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle. MCM has different functions in other species. Defects in MUT are the cause of methylmalonic aciduria type mut (MMAM). MMAM is an often fatal disorder of organic acid metabolism. Common clinical features include lethargy, vomiting, failure to thrive, hypotonia, neurological deficit and early death. Two forms of the disease are distinguished by the presence (mut-) or absence (mut0) of residual enzyme activity. Mut0 patients have more severe neurological manifestations of the disease than do MUT- patients. MMAM is unresponsive to vitamin B12 therapy. Belongs to the methylmalonyl-CoA mutase family.Protein type: Carbohydrate Metabolism - propanoate; EC 5.4.99.2; Isomerase; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradationCellular Component: mitochondrionMolecular Function: catalytic activity; cobalamin binding; intramolecular transferase activity; isomerase activity; metal ion binding; methylmalonyl-CoA mutase activityBiological Process: homocysteine metabolic process; metabolic process; post-embryonic development |
| UniProt Protein Details: | |
| NCBI Summary: | |
| UniProt Code: | P16332 |
| NCBI GenInfo Identifier: | 148540106 |
| NCBI Gene ID: | 17850 |
| NCBI Accession: | NP_032676.2 |
| UniProt Secondary Accession: | P16332,Q3UJU1 |
| UniProt Related Accession: | P16332 |
| Molecular Weight: | 80.5 kDa |
| NCBI Full Name: | methylmalonyl-CoA mutase, mitochondrial |
| NCBI Synonym Full Names: | methylmalonyl-Coenzyme A mutase |
| NCBI Official Symbol: | Mut |
| NCBI Official Synonym Symbols: | Mcm; D230010K02Rik |
| NCBI Protein Information: | methylmalonyl-CoA mutase, mitochondrial |
| UniProt Protein Name: | Methylmalonyl-CoA mutase, mitochondrial |
| UniProt Synonym Protein Names: | Methylmalonyl-CoA isomerase |
| Protein Family: | Methylmalonyl-CoA mutase |
| UniProt Gene Name: | Mut |
| UniProt Entry Name: | MUTA_MOUSE |
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