BCKDK
Reactivité: Humain, Souris
WB
Hôte: Lapin
Polyclonal
RB34170
unconjugated
Indications d'application
ELISA: 1/1,000. Western blotting: 1/100 - 1/500. Immunohistochemistry: 1/50 - 1/100. Other applications not tested. Optimal dilutions are dependent on conditions and should be determined by the user.
Restrictions
For Research Use only
Format
Liquid
Concentration
0.25 mg/mL
Buffer
PBS with 0.09 % (W/V) sodium azide
Agent conservateur
Sodium azide
Précaution d'utilisation
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Conseil sur la manipulation
Avoid repeated freezing and thawing.
Stock
4 °C/-20 °C
Stockage commentaire
Store the antibody undiluted at 2-8 °C for one month or (in aliquots) at-20 °C for longer.
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 ubiquitiously 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.Synonyms: BCKD-kinase, BCKDHKIN, Branched-chain alpha-ketoacid dehydrogenase kinase, [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase mitochondrial