BCKDHB Rabbit pAb
Sizes: 20μL, 100μL
Catalogue Numbers: A10533-20, A10533-100
Citations, Manuals and MSDS Available upon request.
Background: This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation and feeding problems. Alternative splicing at this locus results in multiple transcript variants.
Category: Polyclonal Antibodies
Applications: WB, ELISA
Cross-reactivity: Mouse, Rat
Protein Weight: 43kDa
Observed Molecular Weight: 43kDa
Immunogen: Recombinant protein (or fragment)
Species: Human
GeneID: 594
SWISS: P21953
Alternate Names: E1B; BCKDE1B; BCKDH E1-beta; BCKDHB
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, 1:1000 - 1:5000 ELISA, Recommended starting concentration is 1 μg/mL. Please optimize the concentration based on your specific assay requirements.
Research Areas: Cancer, Signal Transduction, Endocrine Metabolism, Mitochondrial metabolism, Mitochondrial markers, Amino acid metabolism.
NCBI Alias: BCKDHB
Research Use Only