BCKDHA Antibody
Product Specifications
Background
The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3) . This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.
NCBI Gene ID
593
Swiss Prot
P12694
Host
Rabbit
Reactivity
Human, Mouse, Rat
Immunogen
Recombinant fusion protein containing a sequence corresponding to amino acids 276-445 of human BCKDHA (NP_000700.1) .
Clonality
Polyclonal
Conjugation
Unconjugated
Type
Primary Antibodies
Field of Research
Other
Purification
Affinity purification
Positive Control
NCI-H460
Concentration
Batch dependent
Buffer
PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Modification
None
Shipping Conditions
Blue Ice
Storage Conditions
Store at -20˚ C. Avoid freeze / thaw cycles.
Calculated Molecular Weight
Observed: 50kDa
Fragment
IgG
Applications Notes
WB: 1:500 - 1:2000
Symbol
BCKDHA
Positive Control 2
LO2
Positive Control 3
DU145
Positive Control 4
NIH/3T3
Positive Control 5
Mouse liver
Positive Control 6
Mouse kidney
NCBI Official Name
Branched chain keto acid dehydrogenase E1, alpha polypeptide
NCBI Organism
Homo sapiens
Other Product Names
BCKDHA, FLJ45695, MSU, MSUD1, OVD1A, BCKDE1A
Tested Applications
WB
Physical Properties
Liquid
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