BCKDHA Rabbit pAb
Sizes: 20μL, 100μL
Catalogue Numbers: A21588-20, A21588-100
Citations, Manuals and MSDS Available upon request.
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.
Category: Polyclonal Antibodies
Applications: WB, IF/ICC, ELISA
Cross-reactivity: Human, Mouse, Rat
Protein Weight: 50kDa
Observed Molecular Weight: 50kDa
Immunogen: Recombinant protein (or fragment)
Species: Human
GeneID: 593
SWISS: P12694
Alternate Names: MSU; MSUD1; OVD1A; BCKDE1A; BCKDHA
Source: Rabbit
Isotype: IgG
Purity: Affinity purification
Storage: Store at -20℃. Avoid freeze / thaw cycles. Buffer: PBS containing 50% glycerol, preserved with proclin300 or sodium azide (as specified on the Certificate of Analysis), pH 7.3.
Recommended Dilutions: WB, 1:500 - 1:1000 IF/ICC, 1:50 - 1:200 ELISA, Recommended starting concentration is 1 μg/mL. Please optimize the concentration based on your specific assay requirements.
NCBI Alias: BCKDHA
Research Use Only