ELISA: 1/1,000. Western blotting: 1/100 - 1/500. Flow cytometry. Other applications not tested. Optimal dilutions are dependent on conditions and should be determined by the user.
Beschränkungen
Nur für Forschungszwecke einsetzbar
Format
Liquid
Konzentration
0.25 mg/mL
Buffer
PBS with 0.09 % (W/V) sodium azide
Konservierungsmittel
Sodium azide
Vorsichtsmaßnahmen
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Handhabung
Avoid repeated freezing and thawing.
Lagerung
4 °C/-20 °C
Informationen zur Lagerung
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