BCKDHA (Human) Recombinant Protein (Q01)
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Specification
Product Description
Human BCKDHA partial ORF ( NP_000700, 347 a.a. - 445 a.a.) recombinant protein with GST-tag at N-terminal.
Sequence
SVDEVNYWDKQDHPISRLRHYLLSQGWWDEEQEKAWRKQSRRKVMEAFEQAERKPKPNPNLLFSDVYQEMPAQLRKQQESLARHLQTYGEHYPLDHFDK
Host
Wheat Germ (in vitro)
Theoretical MW (kDa)
36.63
Preparation Method
Purification
Glutathione Sepharose 4 Fast Flow
Quality Control Testing
12.5% SDS-PAGE Stained with Coomassie Blue.
Storage Buffer
50 mM Tris-HCI, 10 mM reduced Glutathione, pH=8.0 in the elution buffer.
Storage Instruction
Store at -80°C. Aliquot to avoid repeated freezing and thawing.
Note
Best use within three months from the date of receipt of this protein.
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Applications
Enzyme-linked Immunoabsorbent Assay
Western Blot (Recombinant protein)
Antibody Production
Protein Array
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Gene Info — BCKDHA
Entrez GeneID
593GeneBank Accession#
NM_000709Protein Accession#
NP_000700Gene Name
BCKDHA
Gene Alias
BCKDE1A, FLJ45695, MSU, MSUD1, OVD1A
Gene Description
branched chain keto acid dehydrogenase E1, alpha polypeptide
Gene Ontology
HyperlinkGene 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
Other Designations
2-oxoisovalerate dehydrogenase (lipoamide)|branched-chain alpha-keto acid dehydrogenase complex E1 alpha subunit|maple syrup urine disease
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Interactome
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Pathway
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Disease
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