Thermo Fisher Scientific BCKDH Polyclonal Antibody

Applications

Tested Dilution

Publications

Western Blot (WB)

1:500-1:1,000

-

ELISA (ELISA)

1:10,000

-

Immunoprecipitation (IP)

1:50-1:250

-

Product Specifications

Species Reactivity

Human, Mouse, Rat

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

Synthetic peptide taken within amino acid region 170-200 on human BCKD protein. if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:BCKDK,uniProtId:O14874-1,ncbiNodeId:9606,antigenRange:170-200,antigenLength:412,antigenImageFileName:BCKD-101AP_BCKDK_O14874-1_Rabbit.svg,antigenImageFileNamePDP:BCKD-101AP_BCKDK_O14874-1_Rabbit_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

View immunogen .st0{fill:#FFFFFF;} .st1{fill:#1E8AE7;}

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

0.5-1.5 mg/mL

Purification

Affinity chromatography

Storage buffer

proprietary buffer, pH 7.4-7.8, with 30% glycerol, 0.5% BSA

Contains

0.02% sodium azide

Storage conditions

-20°C

Shipping conditions

Ambient (domestic); Wet ice (international)

Target Information

The second major step in the catabolism of the branched-chain amino acids, isoleucine, leucine, and valine, is irreversibly catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD), an inner-mitochondrial enzyme complex composed of 3 catalytic components: a branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). The complex also contains 2 enzymes that regulated the state of activity of the BCKD complex: a kinase (BCKDK), and a phosphorylase. The ubiquitously expressed kinase contains 1 histidine kinase domain. Maple syrup urine disease (MSUD) is a pathology secondary to an enzyme defect in the catabolic pathway of leucine, isoleucine, and valine. Accumulation of these amino acids and their corresponding keto acids results in encephalopathy and progressive neurodegeneration in infants not treated for MSUD.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.