Anti-DBT Antibody (CAB20381)

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SKU:
CAB20381
Product Type:
Antibody
Antibody Type:
Polyclonal Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Synonyms:
E2
Synonyms:
E2B
Synonyms:
BCATE2
Synonyms:
BCKADE2
Synonyms:
BCKAD-E2
Synonyms:
BCKDH-E2
Synonyms:
BCOADC-E2

Description

Product Name:DBT Rabbit pAb
Product Code:CAB20381
Size:50uL, 100uL
Synonyms:E2, E2B, BCATE2, BCKADE2, BCKAD-E2, BCKDH-E2, BCOADC-E2
Applications:WB, IHC, IF
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant protein of human DBT.
Applications:WB, IHC, IF
Recommended Dilutions:WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:293T, HepG2, A375, Mouse lung, Mouse brain, Rat liver, Rat brain
Immunogen:Recombinant protein of human DBT.
Purification Method:Affinity purification
Storage: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:1629
Uniprot:P11182
Calculated MW:53kDa
Observed MW:53KDa
UniProt Protein Function:DBT: 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). Defects in DBT are the cause of maple syrup urine disease type 2 (MSUD2). MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine. Belongs to the 2-oxoacid dehydrogenase family.
UniProt Protein Details:

Protein type:Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 2.3.1.168; Mitochondrial; Transferase

Chromosomal Location of Human Ortholog: 1p31

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

Molecular Function:dihydrolipoyllysine-residue (2-methylpropanoyl)transferase activity

Biological Process: branched chain family amino acid catabolic process

Disease: Maple Syrup Urine Disease

NCBI Summary:The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined. [provided by RefSeq, Jul 2008]
UniProt Code:P11182
NCBI GenInfo Identifier:400668
NCBI Gene ID:1629
NCBI Accession:P11182.3
UniProt Related Accession:P11182
Molecular Weight:Observed Molecular Weight: (Observed)53kD.Predicted Molecular Weight: (Calculated)53kDa.
NCBI Full Name:Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
NCBI Synonym Full Names:dihydrolipoamide branched chain transacylase E2
NCBI Official Symbol:DBT  
NCBI Official Synonym Symbols:E2; E2B; BCATE2; BCKADE2; BCKAD-E2; BCOADC-E2  
NCBI Protein Information:lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
UniProt Protein Name:Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
UniProt Synonym Protein Names:52 kDa mitochondrial autoantigen of primary biliary cirrhosis; Branched chain 2-oxo-acid dehydrogenase complex component E2
UniProt Gene Name:DBT  
UniProt Entry Name:ODB2_HUMAN
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