DBT Antibody, HRP conjugated

Datasheet
Code CSB-PA006530LB01HU
Size US$299
Uniprot No. P11182
Protocols ELISA Protocol
Immunogen Recombinant Human Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial protein (140-259AA)
Raised in Rabbit
Species Reactivity Human
Tested Applications ELISA
Relevance The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component.
Form Liquid
Conjugate HRP
Storage Buffer Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Purification Method >95%, Protein G purified
Isotype IgG
Clonality Polyclonal
Alias Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial (EC 2.3.1.168) (52 kDa mitochondrial autoantigen of primary biliary cirrhosis) (Branched chain 2-oxo-acid dehydrogenase complex component E2) (BCOADC-E2) (Branched-chain alpha-keto acid dehydrogenase complex component E2) (BCKAD-E2) (BCKADE2) (Dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex) (Dihydrolipoamide branched chain transacylase) (Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase), DBT, BCATE2
Immunogen Species Homo sapiens (Human)
Research Area Tags & Cell Markers
Target Names DBT
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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Function The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components
Involvement in disease Maple syrup urine disease 2 (MSUD2)
Subcellular Location Mitochondrion matrix
Protein Families 2-oxoacid dehydrogenase family
Database Links

HGNC: 2698

OMIM: 248600

KEGG: hsa:1629

STRING: 9606.ENSP00000359151

UniGene: Hs.709187

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