Cell Biology Antibodies 14
Anti-BCKDHA Antibody (CAB16774)
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- SKU:
- CAB16774
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Description
| Antibody Name: | Anti-BCKDHA Antibody |
| Antibody SKU: | CAB16774 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 276-445 of human BCKDHA (NP_000700.1). |
| Application: | WB |
| Recommended Dilution: | WB 1:500 - 1:2000 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | Mouse liver, Mouse kidney, Mouse heart, Rat kidney, Rat liver |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 276-445 of human BCKDHA (NP_000700.1). |
| 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: | SEQY RGDG IAAR GPGY GIMS IRVD GNDV FAVY NATK EARR RAVA ENQP FLIE AMTY RIGH HSTS DDSS AYRS VDEV NYWD KQDH PISR LRHY LLSQ GWWD EEQE KAWR KQSR RKVM EAFE QAER KPKP NPNL LFSD VYQE MPAQ LRKQ QESL ARHL QTYG EHYP LDHF DK |
| Gene ID: | 593 |
| Uniprot: | P12694 |
| Cellular Location: | Mitochondrion matrix |
| Calculated MW: | 50kDa |
| Observed MW: | 40kDa |
 | Western blot - BCKDHA Rabbit pAb (CAB16774) |
| Synonyms: | BCKDHA, BCKDE1A, MSU, MSUD1, OVD1A |
| Background: | The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene. |
| UniProt Protein Function: | BCKDH E1-alpha: branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease). Catalyzes the first irreversible and rate-limiting step in leucine oxidation. Its activity is inhibited by serine phosphorylation |
| UniProt Protein Details: | Protein type:EC 1.2.4.4; Oxidoreductase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; Lyase Chromosomal Location of Human Ortholog: 19q13.1-q13.2 Cellular Component: mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix; mitochondrion Molecular Function:alpha-ketoacid dehydrogenase activity; carboxy-lyase activity; protein binding Biological Process: branched chain family amino acid catabolic process; glyoxylate metabolic process Disease: Maple Syrup Urine Disease |
| NCBI Summary: | The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Sep 2009] |
| UniProt Code: | P12694 |
| NCBI GenInfo Identifier: | 548403 |
| NCBI Gene ID: | 593 |
| NCBI Accession: | P12694.2 |
| UniProt Secondary Accession: | P12694,Q16034, Q16472, B4DP47, E7EW46, |
| UniProt Related Accession: | P12694 |
| Molecular Weight: | 50,790 Da |
| NCBI Full Name: | 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial |
| NCBI Synonym Full Names: | branched chain keto acid dehydrogenase E1, alpha polypeptide |
| NCBI Official Symbol: | BCKDHA |
| NCBI Official Synonym Symbols: | MSU; MSUD1; OVD1A; BCKDE1A |
| NCBI Protein Information: | 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial |
| UniProt Protein Name: | 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial |
| UniProt Synonym Protein Names: | Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; BCKDE1A; BCKDH E1-alpha |
| UniProt Gene Name: | BCKDHA |
| UniProt Entry Name: | ODBA_HUMAN |
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