名称：FH

别名：MCL; LRCC; HLRCC; MCUL1

Targets：Disease

描述

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.

Specification

Swissprot：P07954

Gene Accession：BC003108

WB Predicted band size：55 kDa

Host/Isotype：Rabbit IgG

Antibody Type：Primary antibody

Storage：Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.

Species Reactivity：Human, Mouse, Rat

免疫原：Fusion protein of human FH

Formulation：Purified antibody in PBS with 0.05% sodium azide and 50% glycerol.

Application

Application：WB,IHC

WB：1/500-1/2000

IHC：1/50-1/200

ELISA：1/2000-1/5000

宿主：Rabbit