Product Name: BCKDHB Antibody
Species Reactivity: Human
Tested Applications: IF, IHC-P, WB
Applications: For WB starting dilution is: 1:1000For IHC-P starting dilution is: 1:50~100For FACS starting dilution is: 1:10~50For IF starting dilution is: 1:10~50
User Note: Optimal dilutions for each application to be determined by the researcher.
Predicted Molecular Weight: 43 kDa
Immunogen: 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.
Host Species: Rabbit
Purification: This antibody is purified through a protein A column, followed by peptide affinity purification.
Physical State: Liquid
CAS NO.: 57-08-9
Product: 6-Acetamidohexanoic acid
Buffer: Supplied in PBS with 0.09% (W/V) sodium azide.
Concentration: 0.45 mg/ml
Storage Conditions: Store at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
Clonality: Polyclonal
Conjugate: Unconjugated
Alternate Names: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component beta chain, BCKDE1B, BCKDH E1-beta, BCKDHB
Accession NO.: P21953
Protein Ino: 129034
Official Symbol: BCKDHB
Geneid: 594
Background: Branched-chain keto acid dehydrogenase is a multienzymecomplex associated with the inner membrane of mitochondria, andfunctions in the catabolism of branched-chain amino acids. Thecomplex consists of multiple copies of 3 components: branched-chainalpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2)and lipoamide dehydrogenase (E3). This gene encodes the E1 betasubunit, and mutations therein have been associated with maplesyrup urine disease (MSUD), type 1B, a disease characterized by amaple syrup odor to the urine in addition to mental and physicalretardation, and feeding problems.
PubMed ID:http://aac.asm.org/content/53/11/4885.abstract
