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.
Target
BCKDHB
Reactivity
Human
Host
Rabbit
Clonality
Polyclonal
Tested Applications
WB, IHC, IF/ICC, FCM
Recommended dilutions
Optimal dilutions/concentrations should be determined by the end user.
This BCKDHB antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 41-70 amino acids from the N-terminal region of human BCKDHB.
Storage
Aliquot and store at -20 °C. Avoid repeated freeze/thaw cycles.
Swiss Prot
P21953
Gene Symbol
BCKDHB
Buffer
PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.