Background

The protein FH encoded by this gene is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy.

Images

	Western blotting analysis using anti-fumarate hydratase antibody (Cat#64801). Total cell lysates (5 μg) from various cell lines were loaded and separated by SDS-PAGE. The blot was incubated with anti-fumarate hydratase antibody (Cat#64801, 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-fumarate hydratase antibody (Cat#64801). Fumarate hydratase expression in wild-type (WT) and fumarate hydratase (FH) shRNA knockdown (KD) HeLa cells with 5 μg of total cell lysates. Hsp90 α serves as a loading control. The blot was incubated with anti-fumarate hydratase antibody (Cat#64801, 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 Fumarate hydratase expression in HepG2 cells using anti-Fumarate hydratase antibody (Cat#64801, 1:2,000). Green, isotype control; red, Fumarate hydratase.
	Immunocytochemical staining of HepG2 cells with anti-Fumarate hydratase associated protein antibody (Cat#64801, 1:1,000). Nuclei were stained blue with DAPI; Fumarate hydratase associated protein was stained magenta with Alexa Fluor® 647. Images were taken using Leica stellaris 5. Protein abundance based on laser Intensity and smart gain: High. Scale bar, 20 μm.

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