Product Information
Cite ID#EN5516719
TypePolyclonal Antibodies
HostRabbit
Positive controlMouse pancreas
IsotypeIgG
SummaryThis 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.
FormLiquid
Storage BufferBuffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.