Description
BCKDHB Antibody | F54271-0.08ML | Gentaur US, UK & Europe Disrtribition
Family: Primary antibody
Formulation: In 1X PBS, pH 7.4, with 0.09% sodium azide
Format: Purified
Clone: N/A
Host Animal: Rabbit
Clonality: Polyclonal (rabbit origin)
Species Reactivity: Human
Application: WB, IHC-P, IF, FACS
Buffer: N/A
Limitation: This BCKDHB antibody is available for research use only.
Purity: Antigen affinity purified
Description: Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein 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.
Immunogen: A portion of amino acids 41-70 from the human protein were used as the immunogen for the BCKDHB antibody.
Storage: Aliquot the BCKDHB antibody and store frozen at -20 °C or colder. Avoid repeated freeze-thaw cycles.
