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. [provided by RefSeq]

Specificity:

fumarate hydratase

Alternative names:

FMRD; HLRCC; LRCC; MCL; MCUL1

Species reactivity:

Human, Mouse, Rat, Dog

Host:

Mouse

Ig type:

PBS (pH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide.

Immunogen:

Full length human recombinant protein of human FH (NP_000134) produced in HEK293T cell.

Purification & Purity:

Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G)

Molecular weight:

50.2 kDa(Predicted)

Applications:

IF, WB

Usage:

WB 1:2000, IF 1:50~100

Storage:

PBS (pH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide.

Precautions:

For research use only, not for use in diagnostic procedure.

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