FH antibody | MyBioSource MBS831830 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

FH antibody

catalog :

MBS831830

quantity :

0.1 mL

price :

725 USD

clonality :

monoclonal

host :

mouse

conjugate :

nonconjugated

clone name :

[1F10]

reactivity :

human, rat, dog

application :

western blot, immunohistochemistry, immunocytochemistry

more info or order :

MyBioSource product webpage

image

image 1 :

Western Blot analysis of HEK293T cell lysates (5 ug) transfected with either recombinant FH protein (Right) or empty vector (Left) detected with FH antibody

image 2 :

Immunohistochemical analysis of FH protein in paraffin embedded Human Kidney tissue using FH antibody

image 3 :

Immunofluorescent staining of COS7 cells transiently transfected with recombinant FH protein using FH antibody

product information

catalog number :

MBS831830

products type :

Antibody

products full name :

FH antibody

products short name :

[FH]

products name syn :

[Monoclonal FH; Anti-FH; Fumarate Hydratase; Fumarase; HLRCC; LRCC; MCul1]

other names :

[fumarate hydratase, mitochondrial; Fumarate hydratase, mitochondrial; fumarate hydratase, mitochondrial; fumarate hydratase]

products gene name :

[FH]

other gene names :

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

uniprot entry name :

FUMH_HUMAN

clonality :

Monoclonal

isotype :

IgG1

clone :

[1F10]

host :

Mouse

reactivity :

Human, Dog, Rat

sequence length :

510

purity :

FH antibody was purified by affinity chromatography.

form :

Liquid in PBS, pH 7.3, with 1% BSA, 50% glycerol and 0.02% NaN 3

concentration :

1.0 mg/ml

storage stability :

Store at 4°C short term, -20°C long term. Avoid repeated freeze/thaw cycles.

tested application :

IF, IHC, WB

app notes :

IF: 1:100. IHC: 1:50. WB: 1:1000. FLOW: 1:100

image1 heading :

Western Blot (WB)

image2 heading :

Immunohistochemistry (IHC)

image3 heading :

Immunofluorescence (IF)

image4 heading :

Flow Cytometry

image4 description :

Flow Cytometric analysis of HEK293T cells transfected with either recombinant FH protein (red) or empty vector (blue) stained using FH antibody

other info1 :

Biological Significance: FH 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.

other info2 :

Immunogen: Full length human recombinant protein of human FH (NP_000134) produced in HEK293T cell

products categories :

Proteases, Inhibitors, & Enzymes

products description :

Mouse monoclonal FH antibody

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 :

Biosynthesis Of Antibiotics Pathway (1144997); 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)

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

0.1 mL

price1 :

725 USD

more info or order :

MyBioSource product webpage