This gene encodes the alpha 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 alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. 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.

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Flow cytometric analysis of HADHA expression in HepG2 cells using HADHA antibody . Green, isotype control; red, HADHA.

Western blotting analysis using HADHA antibody . Total cell lysates (30 μg) from various cell lines were loaded and separated by SDS-PAGE. The blot was incubated with HADHA antibody and HRP-conjugated goat anti-mouse secondary antibody respectively. Image was developed using NaQ™ ECL Substrate Kit .

Western blotting analysis using HADHA antibody . HADHA expression in wild type (WT) and HADHA shRNA knockdown (KD) HeLa cells with 20 μg of total cell lysates. Hsp90 α serves as a loading control. The blot was incubated with HADHA antibody and HRP-conjugated goat anti-mouse secondary antibody respectively. Image was developed using NaQ™ ECL Substrate Kit .

Validation of HADHA knockdown using flow cytometry. Wild-type(WT, Blue) and knockdown(KD, Green) HeLa cells were stained with HADHA antibody and analyzed using BD flow cytometer.

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