Human BCKDHA Recombinant Protein (RPPB1439)
- SKU:
- RPPB1439
- Product type:
- Recombinant Protein
- Size:
- 5ug
- Species:
- Human
- Target:
- BCKDHA
- Synonyms:
- 2-oxoisovalerate dehydrogenase subunit alpha mitochondrial
- Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain
- BCKDE1A
- BCKDH E1-alpha
- Source:
- Escherichia Coli
- Uniprot:
- P12694
Description
| Product Name: | Human BCKDHA Recombinant Protein |
| Product Code: | RPPB1439 |
| Size: | 5µg |
| Species: | Human |
| Target: | BCKDHA |
| Synonyms: | 2-oxoisovalerate dehydrogenase subunit alpha mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain, BCKDE1A, BCKDH E1-alpha, BCKDHA, MSU, MSUD1, OVD1A, FLJ45695. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered colorless solution. |
| Formulation: | The BCKDHA solution (0.25 mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 5mM DTT, 30% glycerol and 0.2M NaCl. |
| Stability: | Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles. |
| Purity: | Greater than 80.0% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MGSSHHHHHH SSGLVPRGSH MSSLDDKPQF PGASAEFIDK LEFIQPNVIS GIPIYRVMDR QGQIINPSED PHLPKEKVLK LYKSMTLLNT MDRILYESQR QGRISFYMTN YGEEGTHVGS AAALDNTDLV FGQYREAGVL MYRDYPLELF MAQCYGNISD LGKGRQMPVH YGCKERHFVT ISSPLATQIP QAVGAAYAAK RANANRVVIC YFGEGAASEG DAHAGFNFAA TLECPIIFFC RNNGYAISTP TSEQYRGDGI AARGPGYGIM SIRVDGNDVF AVYNATKEAR RRAVAENQPF LIEAMTYRIG HHSTSDDSSA YRSVDEVNYW DKQDHPISRL RHYLLSQGWW DEEQEKAWRK QSRRKVMEAF EQAERKPKPN PNLLFSDVYQ EMPAQLRKQQ ESLARHLQTY GEHYPLDHFD K |
Branched-chain a-keto acid dehydrogenase E1 component a chain (BCKDHA) is a member of the BCKDHA family. The BCKD (branched-chain alpha-keto acid dehydrogenase) complex is an inner mitochondrial enzyme complex which catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. This complex consists of 3 catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). Mutations in the BCKDHA gene result in maple syrup urine disease, type IA.
BCKDHA Human Recombinant fused with a 21 amino acid His tag at N-terminus produced in E.Coli is a single, non-glycosylated, polypeptide chain containing 421 amino acids (46-445 a.a.) and having a molecular mass of 47.8kDa. The BCKDHA is purified by proprietary chromatographic techniques.
| 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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