FH Antibody | MyBioSource MBS850725 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

FH Antibody

catalog :

MBS850725

quantity :

0.05 mg

price :

225 USD

clonality :

monoclonal

host :

mouse

conjugate :

nonconjugated

reactivity :

human

application :

western blot, ELISA, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS850725

products type :

Antibody

products full name :

FH Antibody

products short name :

other names :

Homo sapiens fumarate hydratase (FH), nuclear gene encoding mitochondrial protein, mRNA; Fumarate hydratase, mitochondrial; fumarate hydratase, mitochondrial; fumarase; fumarate hydratase

products gene name :

other gene names :

FH; FH; MCL; LRCC; HLRCC; MCUL1; Fumarase

uniprot entry name :

FUMH_HUMAN

clonality :

Monoclonal

host :

Mouse

reactivity :

Human

sequence length :

1791

purity :

Protein G-Sepharose affinity purified.

form :

Liquid

concentration :

50ug/50ul

storage stability :

Aliquot and store at -20 degree C. Avoid repeated freeze/thaw cycles

tested application :

ELISA (EIA), Western Blot (WB)

app notes :

ELISA: 1/5000 - 1/20000, WB: 1/100-1/2000

other info1 :

Relevance: FH (Fumarate hydratse) catalyzes the conversion of fumarate to malate. It is a key enzyme in the tricarboxylic acid (TCA) cycle. It exists in a cytosolic form and an N terminal extended form. The N terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. FH also acts as a tumor suppressor. Perservative: 0.02% Sodium Azide and 50% glycerol, 1% BSA, PBS, pH7.4.

other info2 :

Immunogen: Recombinant fragment of human FH (C-terminus). Cellular Localization: Mitochondrion. Pathway: tricarboxylic acid (TCA) cycle

products categories :

Cancer; Cardiovascular; Cell Biology; Epigenetics & Nuclear Signaling; Developmental Biologys; Immunology; Drug Discovery Products; Metabolism; Neuroscience; Signal Transduction; Stem Cells; Autophagy antibody

products description :

Background: Mouse monoclonal IgG to FH

ncbi gi num :

19743874

ncbi acc num :

NP_000134.2

ncbi gb acc num :

NM_000143.2

ncbi mol weight :

50,213 Da

ncbi pathways :

Carbon Metabolism Pathway (814926); Carbon Metabolism Pathway (817567); Citrate Cycle (TCA Cycle) Pathway (82927); Citrate Cycle (TCA Cycle) Pathway (288); Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway (855811); Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway (468202); Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate = Oxaloacetate Pathway (413348); Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate = Oxaloacetate Pathway (468204); Citric Acid Cycle (TCA Cycle) Pathway (105919); Metabolic Pathways (132956)

ncbi summary :

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]

uniprot summary :

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 binding sites: 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; Mitochondrial; EC 4.2.1.2; Tumor suppressor; Lyase. 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

size1 :

0.05 mg

price1 :

225 USD

size2 :

0.1 mg

price2 :

290

more info or order :

MyBioSource product webpage