HADHB (Trifunctional enzyme subunit beta (mitochondrial), Acetyl-CoA acyltransferase) is a 474 amino acid protein encoded by the human gene HADHB. HADHB belong to the thiolase family, which are ubiquitous enzymes that catalyze the reversible thiolytic cleavage of 3-ketoacyl-CoA into acyl-CoA and acetyl-CoA, a 2-step reaction involving a covalent intermediate formed with a catalytic cysteine. HADHB is found in the mitochondrion as an octamer of 4 a (HADHA) and 4 b (HADHB) subunits. Defects in HADHB are a cause of trifunctional protein deficiency (TFP deficiency). The clinical manifestations are very variable and include hypoglycemia, cardiomyopathy and sudden death. Phenotypes with mainly hepatic and neuromyopathic involvement can also be distinguished. Biochemically, TFP deficiency is defined by the loss of all three enzyme activities of the TFP complex.
Key Feature
Clonality
Polyclonal
Isotype
IgG
Host Species
Rabbit
Tested Applications
WBIPIFELISA
recommended for detection of HADHB of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine and bovine
Species Reactivity
HumanMouseRat
Concentration
1mg/ml
Purification
Affinity purified
Target Information
Alternative Names
epitope corresponding to amino acids 1-290 mapping at the N-terminus of HADHB of human origin
Tissue Specificity
epitope corresponding to amino acids 1-290 mapping at the N-terminus of HADHB of human origin
Western blot analysis of HADHB expression in Jurkat whole cell lysate.
Application Notes
recommended for detection of HADHB of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine and bovine
Additional Information
Form
Liquid
Storage Instructions
For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.