Product & ReviewsAntibodies
BCKDHB Rabbit pAb
Product Details
- Cat. No.
- A10533
- Type
- Primary Antibody
- Clonality
- Polyclonal
- Host
- Rabbit
Western blot - BCKDHB antibody (A10533)
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Description
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.
Biological Information
- Clonality: Polyclonal
- Host: Rabbit
- Reactivity: Mouse
