Background

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.

Images

	Western blotting analysis using anti-HADHA antibody (Cat#63587). Total cell lysates (30 μg) from various cell lines were loaded and separated by SDS-PAGE. The blot was incubated with anti-HADHA antibody (Cat#63587, 1:2,500) and HRP-conjugated goat anti-mouse secondary antibody (Cat#101, 1:20,000) respectively. Image was developed using NaQ™ ECL Substrate Kit (Cat#716).
	Western blotting analysis using anti-HADHA antibody (Cat#63587). HADHA expression in wild type (WT) and HADHA shRNA knockdown (KD) HeLa cells with 20 μg of total cell lysates. β-Tubulin serves as a loading control. The blot was incubated with anti-HADHA antibody (Cat#63587, 1:2,500) and HRP-conjugated goat anti-mouse secondary antibody (Cat#101, 1:20,000) respectively. Image was developed using NaQ™ ECL Substrate Kit (Cat#716).
	Flow cytometric analysis of HADHA expression in HepG2 cells using anti-HADHA antibody (Cat#63587, 1:2,000). Green, isotype control; red, HADHA.

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