Target Synonym: FH;FMRD;HLRCC;LRCC;MCL;MCUL1;fumarate hydratase;Fumarase;Fumarate hydratase mitochondrial;FUMH;MCUL 1;mitochondrial;MS709;Multiple hereditary cutaneous leiomyomata;FMRD;fumarate hydratase;HLRCC;LRCC;MCL;MCUL1
Background: The protein 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.
Immunogen: Recombinant Human Fumarate Hydratase / FH protein
Buffer: 0.2 μm filtered solution in PBS
Dilution: WB 1:500-1:1000;
Calculated MW: 55 kDa
Observed MW: 47 kDa
English
Deutsch
