RM2236

FH (11E3) Rabbit Monoclonal Antibody

MCL; FMRD; LRCC; HLRCC; MCUL1

常规抗体

WB

Human,Mouse,Rat

Supplied in 50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40%Glycerol, 0.01% New type preservative N and 0.05% BSA.

Recombinant protein of human FH

WB: 1:1000 IHC: 1:100 ICC/IF: 1:20 FC: 1:100 IP: 1:20

49kDa

55kDa

-20°C/1 year

Rabbit

IgG

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, Jul 2008]

Affinity Purification