Goat anti-Fumarase/FH Antibody | MyBioSource MBS421119 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Goat anti-Fumarase/FH Antibody

catalog :

MBS421119

quantity :

0.1 mg

price :

300 USD

clonality :

polyclonal

host :

goat

conjugate :

nonconjugated

reactivity :

human, mouse, rat, dog

application :

western blot, ELISA, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS421119

products type :

Antibody

products full name :

Goat anti-Fumarase/FH Antibody

products short name :

Fumarase/FH

products name syn :

Goat Anti-Fumarase / FH, Biotinylated Antibody; fumarate hydratase; HLRCC; LRCC; MCL; MCUL1; fumarase; leiomyomatosis and renal cell cancer; multiple hereditary cutaneous leiomyomata; fumarate hydratase antibody; HLRCC antibody; LRCC antibody; MCL antibody; MCUL1 antibody; fumarase antibody; leiomyomatosis and renal cell cancer antibody; multiple hereditary cutaneous leiomyomata antibody; Fumarase; Fumarase / FH

other names :

fumarate hydratase, mitochondrial; Fumarate hydratase, mitochondrial; fumarate hydratase, mitochondrial; fumarate hydratase

products gene name :

other gene names :

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

uniprot entry name :

FUMH_HUMAN

clonality :

Polyclonal

host :

Goat

reactivity :

Tested: Human; Expected from sequence similarity: Human, Mouse, Rat, Dog

sequence length :

510

sequence :

HPNDHVNKSQSSND

purity :

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide

form :

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

concentration :

100ug specific antibody in 200ul

storage stability :

Aliquot and store at -20 degree C. Minimize freezing and thawing.

tested application :

Peptide ELISA (EIA), Western Blot (WB), ELISA (EIA)

app notes :

Peptide ELISA: Antibody detection limit dilution 1: 64000. Western Blot: Approx 48kDa band observed in Human Kidney, Liver and Lung lysates (calculated MW of 54.6kDa according to NP_000134.2). The observed molecular weight corresponds to earlier findings with antibodies from different sources. In transfected HEK293 transiently expressing Fumarase a band of approx.55kDa is observed. This band is not observed in the non-transfected HEK293. Recommended concentration: 0.01-0.03ug/ml. Enzyme immunoassay: Sandwich-type ELISA with increasing amount of recombinant Fumarase captured by a rabbit antibody. Recommended reporter concentration: 1-2ug/ml.

other info1 :

Immunogen: Peptide with sequence C-HPNDHVNKSQSSND, from the internal region of the protein sequence according to NP_000134.2. Epitope: Internal region

ncbi gi num :

19743875

ncbi acc num :

NP_000134.2

ncbi gb acc num :

NM_000143.3

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 (1270125); 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: Tumor suppressor; Lyase; EC 4.2.1.2; Carbohydrate Metabolism - citrate (TCA) cycle; Mitochondrial. Chromosomal Location of Human Ortholog: 1q42.1. Cellular Component: cytoplasm; cytosol; mitochondrial matrix; mitochondrion; tricarboxylic acid cycle enzyme complex. Molecular Function: fumarate hydratase activity; protein binding. Biological Process: fumarate metabolic process; homeostasis of number of cells within a tissue; malate metabolic process; protein tetramerization; tricarboxylic acid cycle. Disease: Fumarase Deficiency

size1 :

0.1 mg

price1 :

300 USD

more info or order :

MyBioSource product webpage