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BCKDK Antibody

CAT: 0223-62-669Size: 400 µLDry Ice: NoHazardous: No
CAT#:0223-62-669Size:400 µL
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AVAILABILITY: InStock
24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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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

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