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anti-FH antibody :: Mouse Fumarase Monoclonal Antibody

Scan QR to view Datasheet Catalog #    MBS9400007
Unit / Price
0.05 mL  /  $200 +1 FREE 8GB USB
0.1 mL  /  $255 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Fumarase (FH), Monoclonal Antibody

 Also Known As   

Fumarase Mouse Monoclonal Antibody(7F1)

 Product Synonym Names    FH; Fumarase; fumarate hydratase; Fumarate hydratase, mitochondrial; FUMH; HLRCC; LRCC; MCL; MCUL1
 Product Gene Name   

anti-FH antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    136850
 3D Structure    ModBase 3D Structure for P07954
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 Clonality    Monoclonal
 Clone Number    7F1
 Host    Mouse
 Species Reactivity    Human, Rat, Mouse
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 Specificity    FH Mouse Monoclonal antibody detects endogenous FH proteins.
 Purity/Purification    Affinity purification using immunogen.
 Form/Format    Mouse IgG1 in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
 Concentration    1.0 mg/ml (lot specific)
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 Target Name    Fumarase
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 Preparation and Storage    Store at -20 degree C
 Other Notes    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.
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Product Description specifically for anti-FH antibody

   Fumarase (FH) is an enzyme that catalyzes the reversible hydration/dehydration of fumarate to malate. Fumarase comes in two forms: mitochondrial and cytosolic. The mitochondrial isoenzyme is involved in the Krebs Cycle (also known as the Tricarboxylic Acid Cycle [TCA] or the Citric Acid Cycle), and the cytosolic isoenzyme is involved in the metabolism of amino acids and fumarate. Subcellular localization is established by the presence of a signal sequence on the amino terminus in the mitochondrial form, while subcellular localization in the cytosolic form is established by the absence of the signal sequence found in the mitochondrial variety.
 Product Categories/Family for anti-FH antibody    Total protein Ab
 Applications Tested/Suitable for anti-FH antibody   

Western Blot (WB)

 Application Notes for anti-FH antibody    Western blotting: 1:2000~1:5000
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 Western Blot (WB) of anti-FH antibody    Western blot analysis of 1) 293T, 2) HepG2, 3) Hela cells, using diluted at 1:3,000.
anti-FH antibody Western Blot (WB) (WB) image
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 Western Blot (WB) of anti-FH antibody    Western blot analysis of 1) Mouse Brain tissue, 2) Rat Brain tissue,, using diluted at 1:3,000.
anti-FH antibody Western Blot (WB) (WB) image
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NCBI/Uniprot data below describe general gene information for FH. It may not necessarily be applicable to this product.
 NCBI GI #    19743875
 NCBI GeneID    2271
 NCBI Accession #    NP_000134.2 [Other Products]
 NCBI GenBank Nucleotide #    NM_000143.3 [Other Products]
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 UniProt Primary Accession #    P07954 [Other Products]
 UniProt Secondary Accession #    B1ANK7 [Other Products]
 UniProt Related Accession #    P07954 [Other Products]
 Molecular Weight    50,213 Da [Similar Products]
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 NCBI Official Full Name    fumarate hydratase, mitochondrial
 NCBI Official Synonym Full Names    fumarate hydratase
 NCBI Official Symbol    FH [Similar Products]
 NCBI Official Synonym Symbols   
MCL; FMRD; LRCC; HLRCC; MCUL1
[Similar Products]
 NCBI Protein Information    fumarate hydratase, mitochondrial
 UniProt Protein Name    Fumarate hydratase, mitochondrial
 UniProt Gene Name    FH [Similar Products]
 UniProt Synonym Gene Names    Fumarase [Similar Products]
 UniProt Entry Name    FUMH_HUMAN
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 NCBI Summary for FH    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]
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 UniProt Comments for FH    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: EC 4.2.1.2; Tumor suppressor; Mitochondrial; Carbohydrate Metabolism - citrate (TCA) cycle; 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; tricarboxylic acid cycle; homeostasis of number of cells within a tissue

Disease: Fumarase Deficiency
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 Research Articles on FH    1. These findings 1) confirm that germline FH mutations may present, albeit rarely with pheochromocytoma or paraganglioma; and 2) extend the clinical phenotype associated with FH mutations to pediatric pheochromocytoma.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with anti-FH antibodyPathways associated with anti-FH antibody
 Reference Product  PubMed Publications
 SDHB antibody  >23 publications with FH and SDHB
 SDHD antibody  >14 publications with FH and SDHD
 SDHC antibody  >14 publications with FH and SDHC
 SDHA antibody  >11 publications with FH and SDHA
 MDH2 antibody  >4 publications with FH and MDH2
 MDH1 antibody  >2 publications with FH and MDH1
 ME1 antibody  >1 publications with FH and ME1
 Products by Pathway  Pathway Diagram
 Biosynthesis Of Antibiotics Pathway antibodies  Biosynthesis Of Antibiotics Pathway Diagram
 Carbon Metabolism Pathway antibodies  Carbon Metabolism Pathway Diagram
 Carbon Metabolism Pathway antibodies  Carbon Metabolism Pathway Diagram
 Citrate Cycle (TCA Cycle) Pathway antibodies  Citrate Cycle (TCA Cycle) Pathway Diagram
 Citrate Cycle (TCA Cycle) Pathway antibodies  Citrate Cycle (TCA Cycle) Pathway Diagram
 Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway antibodies  Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway Diagram
 Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway antibodies  Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway Diagram
 Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate => Oxaloacetate Pathway antibodies  Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate => Oxaloacetate Pathway Diagram
 Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate => Oxaloacetate Pathway antibodies  Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate => Oxaloacetate Pathway Diagram
 Citric Acid Cycle (TCA Cycle) Pathway antibodies  Citric Acid Cycle (TCA Cycle) Pathway Diagram
Diseases associated with anti-FH antibodyOrgans/Tissues associated with anti-FH antibody
 Disease Name  Pubmed Publications
 Dyslipidemias Antibodies  >1818 publications with FH and Dyslipidemias
 Heart Diseases Antibodies  >524 publications with FH and Heart Diseases
 Nervous System Diseases Antibodies  >368 publications with FH and Nervous System Diseases
 Kidney Diseases Antibodies  >304 publications with FH and Kidney Diseases
 Hypertension Antibodies  >284 publications with FH and Hypertension
 Brain Diseases Antibodies  >234 publications with FH and Brain Diseases
 Death Antibodies  >215 publications with FH and Death
 Inflammation Antibodies  >144 publications with FH and Inflammation
 Adenocarcinoma Antibodies  >108 publications with FH and Adenocarcinoma
 HEREDITARY LEIOMYOMATOSIS AND RENAL CELL CANCER Antibodies  >105 publications with FH and HEREDITARY LEIOMYOMATOSIS AND RENAL CELL CANCER
 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >2281 publications with FH and Blood
 Heart Antibodies  >838 publications with FH and Heart
 Vascular Antibodies  >512 publications with FH and Vascular
 Liver Antibodies  >352 publications with FH and Liver
 Bone Antibodies  >333 publications with FH and Bone
 Kidney Antibodies  >302 publications with FH and Kidney
 Brain Antibodies  >297 publications with FH and Brain
 Skin Antibodies  >224 publications with FH and Skin
 Muscle Antibodies  >194 publications with FH and Muscle
 Lung Antibodies  >143 publications with FH and Lung
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