FH, Polyclonal Antibody

Fumarate hydratase; mitochondrial; Fumarase; FH

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This FH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 107-135 amino acids from the N-terminal region of human FH.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.35 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-FH antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

Cancer; Metabolism; Signal Transduction

Western Blot (WB), ELISA (EIA)

WB~~1:1000

FH Antibody (N-term) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the FH Antibody detected the FH protein (arrow).

54637

fumarate hydratase

fumarate hydratase, mitochondrial

Fumarate hydratase, mitochondrial

Fumarase  [Similar Products]

FUMH_HUMAN

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]

FH: a metabolic enzyme that participates in the tricarboxylic acid cycle that catalyzes the conversion of (S)-malate into fumarate + H2O. There are two substrate s: the catalytic A site, and the non-catalytic B site that may play a role in the transfer of substrate or product between the active site and the solvent. Alternatively, the B site may bind allosteric effectors. Fumarate accumulates in the cell when FH is inactivated. Fumarate inhibits the dioxygenases that hydroxylate the transcription factor HIF and leads to its degradation by VHL. Since HIF turns on oncogenic pathways, FH has apparent tumor suppressor activity. Defects in FH are the cause of hereditary leiomyomatosis and renal cell cancer (HLRCC), a highly metastatic form of RCC. Defects in FH are the cause of fumarase deficiency (FD) also known as fumaricaciduria. FD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia. Cells derived from a patient with HLRCC exhibit compromised oxidative phosphorylation, dependence on anaerobic glycolysis, rapid glycolytic flux, and overexpression of lactate dehydrogenase A (LDHA) and GLUT1. Two human isoforms are produced by alternative initiation. The longer isoform is mitochondrial, while the shorter form, missing residues 1-43, is cytoplasmic.

Protein type: Carbohydrate Metabolism - citrate (TCA) cycle; Lyase; Tumor suppressor; Mitochondrial; EC 4.2.1.2

Chromosomal Location of Human Ortholog: 1q42.1

Cellular Component: tricarboxylic acid cycle enzyme complex; mitochondrion; mitochondrial matrix; cytoplasm

Molecular Function: fumarate hydratase activity

Biological Process: cellular metabolic process; fumarate metabolic process; homeostasis of number of cells within a tissue; tricarboxylic acid cycle

Disease: Fumarase Deficiency

Shimada, M., et al. Hum. Genet. 128(4):433-441(2010)
Allegri, G., et al. J. Inherit. Metab. Dis. 33(4):411-419(2010)
Yogev, O., et al. PLoS Biol. 8 (3), E1000328 (2010) :
Yang, Y., et al. Cancer Genet. Cytogenet. 196(1):45-55(2010)
Rikova, K., et al. Cell 131(6):1190-1203(2007)

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