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Invitrogen
BCKDHA Recombinant Rabbit Monoclonal Antibody (HL2405)
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Product Details
MA5-50519
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human,
Mouse,
Zebrafish
Host/Isotype
Rabbit
/ IgG
Expression System
HEK293 cells
Class
Recombinant Monoclonal
Type
Antibody
Clone
HL2405
Immunogen
Recombinant fragment of human BCKDHA.
Conjugate
Form
Liquid
Concentration
1 mg/mL
Amount
100 µg
Purification
Protein A
Storage buffer
PBS
Contains
no preservative
Storage conditions
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Shipping conditions
Ambient (domestic); Wet ice (international)
RRID
Target Information
The BCKDHA gene encodes the E1-alpha subunit of the branched-chain alpha-keto acid (BCAA) dehydrogenase complex (BCKD; EC 1.2.4.4), an inner-mitochondrial enzyme complex that catalyzes the oxidative decarboxylation of the branched-chain alpha-ketoacids derived from isoleucine, leucine, and valine. This reaction is the second major step in the catabolism of the branched-chain amino acids (Wynn et al., 1998). The BCKD complex consists of 3 catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a homo-24-meric dihydrolipoyl transacylase (E2), and a homodimeric dihydrolipoamide dehydrogenase (E3). E1 is a thiamine pyrophosphate (TPP)-dependent enzyme. The reaction is irreversible and constitutes the first committed step in BCAA oxidation. The BCKDHB gene encodes the beta subunit of E1. The complex also contains 2 regulatory enzymes, a kinase and a phosphorylase.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: 2-oxoisovalerate dehydrogenase (lipoamide); 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; BCKAD E1[a]; BCKDE1A; BCKDH E1-alpha; branched chain keto acid dehydrogenase E1 alpha protein; branched chain keto acid dehydrogenase E1, alpha polypeptide; Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; branched-chain alpha-keto acid dehydrogenase E1-alpha subunit; unnamed protein product
Gene Aliases: BCKDE1A; BCKDHA; MSU; MSUD1; MSUD1A; OVD1A; wu:fd20d04; zgc:110049
UniProt ID: (Human) P12694
Entrez Gene ID: (Human) 593, (Mouse) 12039, (Zebrafish) 554124
3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity
protein binding
oxidoreductase activity
oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
carboxy-lyase activity
metal ion binding
alpha-ketoacid dehydrogenase activity
protein complex binding
dehydrogenase
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Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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