Anti-BCKDHB monoclonal antibody (DCABH-10723) Made to order

Rabbit anti-Human BCKDHB monoclonal antibody for WB, ELISA

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Specifications


Host Species
Rabbit
Antibody Isotype
IgG
Species Reactivity
Human
Immunogen
A synthetic peptide of human BCKDHB is used for rabbit immunization.
Conjugate
Unconjugated

Target


Alternative Names
BCKDHB; branched chain keto acid dehydrogenase E1, beta polypeptide; 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial; maple syrup urine disease; BCKDE1B; BCKDH E1-beta
Entrez Gene ID
UniProt ID

Product Background


Gene summary
BCKDHB (Branched Chain Keto Acid Dehydrogenase E1, Beta Polypeptide) is a Protein Coding gene. Diseases associated with BCKDHB include maple syrup urine disease, type ii and classic maple syrup urine disease. Among its related pathways are Metabolism and Valine, leucine and isoleucine degradation. GO annotations related to this gene include protein complex binding and alpha-ketoacid dehydrogenase activity. An important paralog of this gene is PDHB. This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation and feeding problems. Alternative splicing at this locus results in multiple transcript variants.
Antigen Description
Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems. Clinical features include mental and physical retardation, feeding problems, and a maple syrup odor to the urine. The keto acids of the branched-chain amino acids are present in the urine. Note=The disease is caused by mutations affecting the gene represented in this entry. 2-Oxoisovalerate dehydrogenase subunit beta, mitochondrial is an enzyme that in humans is encoded by the BCKDHB gene. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2), and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B. Alternative splicing at this locus results in transcript variants with different 3 noncoding regions, but encoding the same isoform. The function about BCKDHB antigen include 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity; alpha-ketoacid dehydrogenase activity; carboxy-lyase activity; protein binding; protein complex binding.
Pathway
2-oxobutanoate degradation I, organism-specific biosystem; 2-oxobutanoate degradation I, conserved biosystem; Branched-chain amino acid catabolism, organism-specific biosystem; Leucine degradation, leucine => acetoacetate + acetyl-CoA, organism-specific biosystem; acetoacetate + acetyl-CoA, conserved biosystem.

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We offer labeled antibodies using our catalogue antibody products and a broad range of intensely fluorescent dyes and labels including HRP, biotin, ALP, Alexa Fluor® dyes, DyLight® Fluor dyes, R-phycoerythrin (R-PE), at scales from less than 100 μg up to 1 g of IgG antibody. Learn More

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References


CLARKSON, GHD; LINDSAY, JG; et al. IMMUNOLOGY, BIOSYNTHESIS AND INVIVO ASSEMBLY OF THE BRANCHED-CHAIN 2-OXOACID DEHYDROGENASE COMPLEX FROM BOVINE KIDNEY. EUROPEAN JOURNAL OF BIOCHEMISTRY 196:95-100(1991).

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