Anti-DBT Antibody (CAB20381)
- SKU:
- CAB20381
- Product Type:
- Antibody
- Antibody Type:
- Polyclonal Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Synonyms:
- E2
- Synonyms:
- E2B
- Synonyms:
- BCATE2
- Synonyms:
- BCKADE2
- Synonyms:
- BCKAD-E2
- Synonyms:
- BCKDH-E2
- Synonyms:
- BCOADC-E2
Description
| Product Name: | DBT Rabbit pAb |
| Product Code: | CAB20381 |
| Size: | 50uL, 100uL |
| Synonyms: | E2, E2B, BCATE2, BCKADE2, BCKAD-E2, BCKDH-E2, BCOADC-E2 |
| Applications: | WB, IHC, IF |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant protein of human DBT. |
| Applications: | WB, IHC, IF |
| Recommended Dilutions: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | 293T, HepG2, A375, Mouse lung, Mouse brain, Rat liver, Rat brain |
| Immunogen: | Recombinant protein of human DBT. |
| Purification Method: | Affinity purification |
| Storage: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | Email for sequence |
| Gene ID: | 1629 |
| Uniprot: | P11182 |
| Calculated MW: | 53kDa |
| Observed MW: | 53KDa |
| UniProt Protein Function: | DBT: The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Defects in DBT are the cause of maple syrup urine disease type 2 (MSUD2). MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine. Belongs to the 2-oxoacid dehydrogenase family. |
| UniProt Protein Details: | Protein type:Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 2.3.1.168; Mitochondrial; Transferase Chromosomal Location of Human Ortholog: 1p31 Cellular Component: mitochondrion; mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix Molecular Function:dihydrolipoyllysine-residue (2-methylpropanoyl)transferase activity Biological Process: branched chain family amino acid catabolic process Disease: Maple Syrup Urine Disease |
| NCBI Summary: | The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined. [provided by RefSeq, Jul 2008] |
| UniProt Code: | P11182 |
| NCBI GenInfo Identifier: | 400668 |
| NCBI Gene ID: | 1629 |
| NCBI Accession: | P11182.3 |
| UniProt Related Accession: | P11182 |
| Molecular Weight: | Observed Molecular Weight: (Observed)53kD.Predicted Molecular Weight: (Calculated)53kDa. |
| NCBI Full Name: | Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial |
| NCBI Synonym Full Names: | dihydrolipoamide branched chain transacylase E2 |
| NCBI Official Symbol: | DBT |
| NCBI Official Synonym Symbols: | E2; E2B; BCATE2; BCKADE2; BCKAD-E2; BCOADC-E2 |
| NCBI Protein Information: | lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial |
| UniProt Protein Name: | Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial |
| UniProt Synonym Protein Names: | 52 kDa mitochondrial autoantigen of primary biliary cirrhosis; Branched chain 2-oxo-acid dehydrogenase complex component E2 |
| UniProt Gene Name: | DBT |
| UniProt Entry Name: | ODB2_HUMAN |
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