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.
Applications:Suitable for use in Western Blot, Immunohistochemistry, Immunofluorescence, Flow Cytometry (Not Tested), ELISA
Storage and Stability:Store product at 4°C if to be used immediately within two weeks.
For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C.
Aliquots are stable at -20°C for 12 months after receipt.
Dilute required amount only prior to immediate use.
Further dilutions can be made in assay buffer.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Note: Applications are based on unconjugated antibody.
仕様
Size:200ul
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by Protein A affinity chromatography.
Form:Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.
Specificity:Human
Isotype:IgG
Calc Applications Abbrev:E IF IHC WB
Calc Crossreactivity:Hu
Immunogen:BCKDHB antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 44-74 amino acids from the N-terminal region of human BCKDHB.