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BCKDHA Antibody

CAT: 0247-OAGA03790-100ULSize: 100 µLDry Ice: NoHazardous: No
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CAT#:0247-OAGA03790-100ULSize:100 µL
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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CAS Number
9007-83-4
Gene Name
Branched chain keto acid dehydrogenase E1, alpha polypeptide
Gene Aliases
MSU, MSUD1, OVD1A, BCKDE1A
Gene ID
593
Accession Number
NP_000700.1
Reactivity
Human
Immunogen
Recombinant protein encompassing a sequence within the C-terminus region of human BCKDHA. The exact sequence is proprietary.
Target
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.
Clonality
Polyclonal
Type
Polyclonal Antibody
Applications
ICC, IF, IHC-P, WB
Purification
Purified by antigen-affinity chromatography.
Assay Protocol
Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Reconstitution & Storage Instructions Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Antibody Array (AA) Protocol
Concentration
1 mg/ml
Format
Liquid
Buffer
1XPBS, 1% BSA, 20% Glycerol (pH7) . 0.01% Thimerosal was added as a preservative.
Reconstitution
Keep as concentrated solution. Aliquot and store at -20C or below. Avoid multiple freeze-thaw cycles.
Fragment
IgG
NCBI Gene Symbol
BCKDHA
Host or Source
Rabbit
Protein Name
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Gene Name URL
BCKDHA
Nucleotide Accession Number
NM_000709.3

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