FH Monoclonal Antibody(7F1)

Ab type

Primary antibody

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.

Reactivity

Human,Mouse,Rat

Clonality

Monoclonal

Host

Mouse

Isotype

IgG

Immunogen

Synthetic Peptide of FH

Storage Stability

-20°C for one year

Concentration

1 mg/ml

MolecularWeight_Da

N/A

synonyms

Fumarate hydratase, mitochondrial; Fumarase; EC 4.2.1.2