Thermo Fisher Scientific BCKDK Monoclonal Antibody (OTI11C9)

Applications

Tested Dilution

Publications

Western Blot (WB)

1:2,000

-

Immunohistochemistry (Paraffin) (IHC (P))

1:50

-

Immunocytochemistry (ICC/IF)

1:100

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Mouse / IgG1

Class

Monoclonal

Type

Antibody

Clone

OTI11C9

Immunogen

Full length human recombinant protein of BCKDK produced in HEK293T cell if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:BCKDK,uniProtId:O14874-1,ncbiNodeId:9606,antigenRange:1-412,antigenLength:412,antigenImageFileName:MA5-24992_BCKDK_O14874-1_House_mouse.svg,antigenImageFileNamePDP:MA5-24992_BCKDK_O14874-1_House_mouse_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

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

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

1 mg/mL

Purification

Affinity Chromatography

Storage buffer

PBS, pH 7.3, with 1% BSA, 50% glycerol

Contains

0.02% sodium azide

Storage conditions

-20° C, Avoid Freeze/Thaw Cycles

Shipping conditions

Ambient (domestic); Wet ice (international)

RRID

AB_2722891

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.