Recombinant Human BCKDHA

• Cat.No.: BCKDHA-962H
• Product Overview: Recombinant human BCKDHA protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques.

Specification

• Cat. No.: BCKDHA-962H
• Description: BCKDHA (branched-chain-keto acid dehydrogenase E1 component chain), also known as 2-oxoisovalerate dehydrogenase subunit alpha, belongs to the BCKDHA family. The branched-chain alpha-keto acid dehydrogenase 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).
• Source: E.coli.
• Form: Liquid. In 20mM Tris-HCl buffer (pH 8.0) containing 5mM DTT, 30% glycerol, 0.2M NaCl.
• Molecular Weight: 47.8kDa (421aa) confirmed by MALDI-TOF.
• Purity: > 80% by SDS – PAGE.
• Concentration: 0.25 mg/ml (determined by Bradford assay).
• Sequences Of Amino Acids: MGSSHHHHHH SSGLVPRGSH MSSLDDKPQF PGASAEFIDK LEFIQPNVIS GIPI YRVMDR QGQIINPSED PHLPKEKVLK LYKSMTLLNT MDRILYESQR QGRI SFY MTN YGEEGTHVGS AAALDNTDLV FGQYREAGVL MYRDYPLELF MAQ CYGNISD LGK GRQMPVH YGCKERHFVT ISSPLATQIP QAVGAAYAAK RAN AN RVVIC YF G EGAASEG DAHAGFNFAA TLECPIIFFC RNNGYAISTP TSEQ YRGDGI AA RG PG YGIM SIRVDGNDVF AVYNATKEAR RRAVAENQPF LIEA MTYRIG HH STS DD S SA YRSVDEVNYW DKQDHPISRL RHYLLSQGWW DEE QEKAWRK QS RRK VME A F EQAERKPKPN PNLLFSDVYQ EMPAQLRKQQ ESLA RHLQTY GEHYPLDHFD K.
• Storage: Can be stored at +4°C short term (1-2 weeks). For long term storage, aliquot and store at -20°C or -70°C. Avoid repeated freezing and thawing cycles.
• Pathways: Metabolic pathways; Valine, leucine and isoleucine degradation; Metabolism of amino acids and derivatives
• Protein length: 46-445 a.a.

Gene Information

• Gene Name: BCKDHA branched chain keto acid dehydrogenase E1, alpha polypeptide [ Homo sapiens ]
• Synonyms: branched chain keto acid dehydrogenase E1, alpha polypeptide; MSU; MSUD1; OVD1A; BCKDE1A; FLJ45695; BCKDHA;2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; BCKDH E1-alpha ; 2-oxoisovalerate dehydrogenase (lipoamide) ; branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease); EC 1.2.4.4
• Gene ID: 593
• mRNA Refseq: NM_000709
• Protein Refseq: NP_000700
• MIM: 608348
• UniProt ID: P12694
• Chromosome Location: 19q13.1-q13.2
• Function: 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity; alpha-ketoacid dehydrogenase activity; carboxy-lyase activity; metal ion binding; protein binding