Thermo Fisher Scientific BCKDK Polyclonal Antibody

Applications

Tested Dilution

Publications

Western Blot (WB)

1:500-1:3,000

-

Immunohistochemistry (Paraffin) (IHC (P))

1:100-1:1,000

-

Product Specifications

Species Reactivity

Human, Mouse, Rat

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

Recombinant fragment corresponding to a region within amino acids 46 and 327 of Human BCKDK if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:BCKDK,uniProtId:O14874-1,ncbiNodeId:9606,antigenRange:46-327,antigenLength:412,antigenImageFileName:PA5-31455_BCKDK_O14874-1_Rabbit.svg,antigenImageFileNamePDP:PA5-31455_BCKDK_O14874-1_Rabbit_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

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

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

0.8 mg/mL

Purification

Antigen affinity chromatography

Storage buffer

0.1M tris glycine, pH 7, with 20% glycerol

Contains

0.01% thimerosal

Storage conditions

Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.

Shipping conditions

Wet ice

RRID

AB_2548929

Product Specific Information

Recommended positive controls: 293T, mouse brain, rat brain.

Predicted reactivity: Mouse (99%), Rat (99%), Zebrafish (85%), Xenopus laevis (89%), Rhesus Monkey (98%), Bovine (98%).

Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.

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.

⚠WARNING: This product can expose you to chemicals including mercury, which is known to the State of California to cause birth defects or other reproductive harm. For more information go to www.P65Warnings.ca.gov.

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