BCKDK Antibody

• Background:

  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.

• NCBI Gene ID:

  10295

• Swiss Prot:

  O14874

• Accession Number:

  O14874

• Host:

  Rabbit

• Reactivity:

  Human

• Immunogen:

  This BCKDK antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 120-151 amino acids from the Central region of human BCKDK.

• Clonality:

  Polyclonal

• Conjugation:

  Unconjugated

• Type:

  Primary Antibodies

• Field of Research:

  Obesity, Signal Transduction

• Purification:

  This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis

• Concentration:

  Batch dependent

• Homology:

  Predicted species reactivity based on immunogen sequence: Mouse, Rat

• Buffer:

  Supplied in PBS with 0.09% (W/V) sodium azide.

• Modification:

  None

• Shipping Conditions:

  Blue Ice

• Storage Conditions:

  Store at 4˚ C for three months and -20˚ C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

• Calculated Molecular Weight:

  46 kDa

• Fragment:

  Rabbit Ig

• Symbol:

  BCKDK

• NCBI Official Name:

  [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial

• NCBI Organism:

  Homo sapiens

• Background Reference 01:

  Chang, C.F., et al., J. Biol. Chem. 277 (18) :15865-15873 (2002) .

• Background Reference 02:

  Popov, K.M., et al., J. Biol. Chem. 267 (19) :13127-13130 (1992) .

• Background Reference 03:

  Zneimer, S.M., et al., Genomics 10 (3) :740-747 (1991) .

• Other Product Names:

  [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial, Branched-chain alpha-ketoacid dehydrogenase kinase, BCKD-kinase, BCKDHKIN, BCKDK

• Tested Applications:

  WB, IHC-P

• Physical Properties:

  Liquid