Anti-BCKDK Antibody [JE63-77]

HUABIO

Anti-BCKDK Antibody [JE63-77]

2

Western Blot

Research Use Only

Monoclonal

JE63-77

1 mg/ml

Unconjugated

BCKDK

branched chain keto acid dehydrogenase kinase

BCKDKD, BDK, branched-chain alpha-ketoacid dehydrogenase kinase, BCKD-kinase, BCKDH kinase, BCKDHKIN, [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial, branched chain alpha-ketoacid dehydrogenase kinase, branched chain ketoacid dehydrogenase kinase

Rabbit

IgG

Branched-chain alpha-ketoacid dehydrogenase kinase

Branched chain ketoacid dehydrogenase kinase (BCKDK) is an enzyme encoded by the BCKDK gene on chromosome 16. This enzyme is part of the mitochondrial protein kinases family and it is a regulator of the valine, leucine, and isoleucine catabolic pathways. BCKDK is found in the mitochondrial matrix and the prevalence of it depends on the type of cell. Liver cells tend to have the lowest concentration of BCKDK, whereas skeletal muscle cells have the highest amount. Abnormal activity of this enzyme often leads to diseases such as maple syrup urine disease and cachexia. BCKDK regulates the activity of branched-chain alpha-ketoacid dehydrogenase complex (BCKD) through phosphorylation and inactivation. This inactivation results in increased branched-chain amino acids (BCAA), which is seen to reduce oxidative stress; however, having too much BCAA has been proven to be toxic to humans. Therefore, BCKDK is a vital tool to assist with BCAA homeostasis. As stated earlier, BCKDK concentrations vary depending on the type of tissue that is observed, whereas BCKDs concentration is the same in any tissue. Although BCKD concentration is constant, the amount of BCKDK determines the activity of the dehydrogenase complex. Since liver tissue is seen to have the lowest concentration of BCKDK, the activity of BCKD is seen to be the highest, delineating the fact that the BCKD kinase inversely affects the BCKD activity.

-20°C,2°C to 8°C

41116161