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Anti-BCKDHA Antibody (CAB19962)[KO Validated]

SKU:
CAB19962
Product Type:
Antibody
Reactivity:
Human
Mouse
Rat
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology
€139 - €529
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Description

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Antibody Name:Anti-BCKDHA Antibody [KO Validated]
Antibody SKU:CAB19962
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant protein of human BCKDHA.
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse, Rat
Positive Samples:293T
Immunogen:Recombinant protein of human BCKDHA.
Purification Method:Affinity purification
Storage Buffer:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:593
Uniprot:P12694
Cellular Location:Mitochondrion matrix
Calculated MW:50kDa
Observed MW:45KDa
Anti-BCKDHA Antibody [KO Validated] (CAB19962)Western blot analysis of extracts from normal (control) and BCKDHA knockout (KO) 293T cells, using BCKDHA antibody (CAB19962) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 1s.
Synonyms:BCKDHA, BCKDE1A, MSU, MSUD1, OVD1A
Background: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.
UniProt Protein Function:BCKDH E1-alpha: branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease). Catalyzes the first irreversible and rate-limiting step in leucine oxidation. Its activity is inhibited by serine phosphorylation
UniProt Protein Details:

Protein type:EC 1.2.4.4; Oxidoreductase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; Lyase

Chromosomal Location of Human Ortholog: 19q13.1-q13.2

Cellular Component: mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix; mitochondrion

Molecular Function:alpha-ketoacid dehydrogenase activity; carboxy-lyase activity; protein binding

Biological Process: branched chain family amino acid catabolic process; glyoxylate metabolic process

Disease: Maple Syrup Urine Disease

NCBI 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.[provided by RefSeq, Sep 2009]
UniProt Code:P12694
NCBI GenInfo Identifier:548403
NCBI Gene ID:593
NCBI Accession:P12694.2
UniProt Secondary Accession:P12694,Q16034, Q16472, B4DP47, E7EW46,
UniProt Related Accession:P12694
Molecular Weight:50,790 Da
NCBI Full Name:2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
NCBI Synonym Full Names:branched chain keto acid dehydrogenase E1, alpha polypeptide
NCBI Official Symbol:BCKDHA  
NCBI Official Synonym Symbols:MSU; MSUD1; OVD1A; BCKDE1A  
NCBI Protein Information:2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
UniProt Protein Name:2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
UniProt Synonym Protein Names:Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; BCKDE1A; BCKDH E1-alpha
UniProt Gene Name:BCKDHA  
UniProt Entry Name:ODBA_HUMAN

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