Anti-BCKDHA Antibody (CAB19962)[KO Validated]
![Anti-BCKDHA Antibody (CAB19962)[KO Validated]](https://cdn11.bigcommerce.com/s-d5atsu6k01/images/stencil/608x608/products/22235/22083/anti-bckdha-antibody-cab19962ko-validated__61293__84321.1645805101.jpg?c=1 "Anti-BCKDHA Antibody (CAB19962)[KO Validated]")
- SKU:
- CAB19962
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Cell Biology
![Anti-BCKDHA Antibody (CAB19962)[KO Validated]](https://cdn11.bigcommerce.com/s-d5atsu6k01/images/stencil/1280x1280/products/22235/22083/anti-bckdha-antibody-cab19962ko-validated__61293__84321.1645805101.jpg?c=1&imbypass=on "Anti-BCKDHA Antibody (CAB19962)[KO Validated]")
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Description
| 抗体名: | Anti-BCKDHA Antibody [KO Validated] |
| 抗体コード: | CAB19962 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB |
| 反応性: | Human, Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant protein of human BCKDHA. |
| 申し込み: | WB |
| 推奨希釈: | WB 1:500 - 1:2000 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | 293T |
| 免疫原: | Recombinant protein of human BCKDHA. |
| 精製方法: | Affinity purification |
| ストレージバッファ: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| アイソタイプ: | IgG |
| 順序: | Email for sequence |
| 遺伝子ID: | 593 |
| Uniprot: | P12694 |
| セルラーロケーション: | Mitochondrion matrix |
| 計算された分子量: | 50kDa |
| 観察された分子量: | 45KDa |
![Anti-BCKDHA Antibody [KO Validated] (CAB19962)](https://cdn11.bigcommerce.com/s-h68l9z2lnx/product_images/p/504/A19962_1__27870.jpg) | Western blot analysis of extracts from normal (control) and BCKDHA knockout (KO) 293T cells, using BCKDHA antibody (CAB19962) 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 Basic Kit (RM00020). Exposure time: 1s. |
| 同義語: | BCKDHA, BCKDE1A, MSU, MSUD1, OVD1A |
| バックグラウンド: | 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 |