HADHB is the beta subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. Mutations in HADHB gene result in trifunctional protein deficiency. The protein can also bind RNA and decreases the stability of some mRNAs.This gene encodes the beta subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. Mutations in this gene result in trifunctional protein deficiency. The encoded protein can also bind RNA and decreases the stability of some mRNAs. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. Alternatively spliced transcript variants have been found; however, their full-length nature is not known. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.1) Wang, R., (2006) Zhonghua Fu Chan Ke Za Zhi 41 (10), 672-675.
Immunogen
Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human HADHB.
Key Feature
Clonality
Polyclonal
Host Species
Rabbit
Tested Applications
ELISAIHCWB
HADHB antibody can be used for detection of HADHB by ELISA at 1:312500. HADHB antibody can be used for detection of HADHB by western blot at 0.25 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.
HADHB antibody can be used for detection of HADHB by ELISA at 1:312500. HADHB antibody can be used for detection of HADHB by western blot at 0.25 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.
Additional Information
Form
Liquid
Storage Instructions
For short periods of storage (days) store at 4˚C. For longer periods of storage, store HADHB antibody at -20˚C. As with any antibody avoid repeat freeze-thaw cycles.
Storage Buffer
Antibody is lyophilized in PBS buffer with 2% sucrose. Add 50 μL of distilled water. Final antibody concentration is 1 mg/mL.