Mouse anti-Human FH/Fumarate Hydratase Monoclonal Antibody | anti-FH antibody

Anti-FH/Fumarate Hydratase Antibody, Mouse Monoclonal

Cat. Num. AAA8106188

• Gene Names: FH; MCL; LRCC; HLRCC; MCUL1
• Reactivity: Human
• Applications: Western Blot, ELISA, Immunoprecipitation
• Purity: Protein A
• Synonyms: FH/Fumarate Hydratase; Monoclonal Antibody; Anti-FH/Fumarate Hydratase Antibody; Mouse Monoclonal; Fumarate Hydratase/FH Antibody; Mouse MAb; fumarate hydratase; Anti-FMRD Antibody; Anti-HLRCC Antibody; Anti-LRCC Antibody; Anti-MCL Antibody; Anti-MCUL1 Antibody; anti-FH antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: Mouse IgG1
• Clone Number: 3D5A3G7
• Specificity: Human Fumarate Hydratase/FH.; No cross-reactivity in ELISA with E Coli cell lysate
• Purity/Purification: Protein A
• Form/Format: Liquid; 0.2um filtered solution in PBS

• Applicable Applications for anti-FH antibody: Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)
• Application Notes: WB: 1:500-1:1000; ELISA: 1:5000-1:10,000; IP: 0.2-1uL/mg of lysate
• Immunogen: Recombinant Human Fumarate Hydratase/FH protein
• Conjugation: Unconjugated
• Preparation: This antibody was produced from a hybridoma resulting from the fusion of a mouse myeloma with B cells obtained from a mouse immunized with purified, recombinant Human Fumarate Hydratase/FH (rh Fumarate Hydratase/FH). The IgG fraction of the cell culture supernatant was purified by Protein A affinity chromatography.
• Preparation and Storage: This antibody can be stored at 2-8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Preservative-Free. Avoid repeated freeze-thaw cycles.

Western Blot (WB)

(Anti-FH mouse monoclonal antibody at 1:500 dilutionLane A: Hela Whole Cell LysateLane B: 293T Whole Cell LysateLysates/proteins at 30 ug per lane.SecondaryGoat Anti-Mouse IgG H&L (Dylight800) at 1/15000 dilution.Developed using the Odyssey technique. Performed under reducing conditions.Predicted band size:55 kDaObserved band size:47 kDa)

Immunoprecipitation (IP)

(FH was immunoprecipitated using:Lane A:0.5 mg Hela Whole Cell LysateLane B:0.5 mg 293T Whole Cell LysateLane C:0.5 mg A431 Whole Cell Lysate0.5 uL anti-FH mouse monoclonal antibody and 15 ul of 50 % Protein G agarose.Primary antibody:Anti-FH mouse monoclonal antibody,at 1:250 dilution Secondary antibody:Dylight 800-labeled antibody to Mouse IgG (H+L), at 1:7500 dilutionDeveloped using the odssey technique.Performed under reducing conditions.Predicted band size: 55 kDaObserved band size: 48 kDa)

Related Product Information for anti-FH antibody

Fumarate Hydratase (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. Fumarate Hydratase 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. Individuals with hemizygous germline fumarate hydratase (FH) mutations are predisposed to renal cancer. These tumors predominantly exhibit functional inactivation of the remaining wild-type allele, implicating FH inactivation as a tumor-promoting event.

References

King A, et al. (2005) Succinate dehydrogenase and fumarate hydratase: linking mitochondrial dysfunction and cancer. Oncogene. 25(34): 4675-82.Alam NA, et al. (2003) Genetic and functional analyses of FH mutations in multiple cutaneous and uterine leiomyomatosis, hereditary leiomyomatosis and renal cancer, and fumarate hydratase deficiency. Hum Mol Genet.12(11): 1241-52.Pollard PJ, et al. (2003) The TCA cycle and tumorigenesis: the examples of fumarate hydratase and succinate dehydrogenase. Ann Med. 35(8): 632-9.

NCBI and Uniprot Product Information

• NCBI GI #: 19743875
• NCBI GeneID: 2271
• NCBI Accession #: NP_000134.2
• NCBI GenBank Nucleotide #: NM_000143.3
• UniProt Accession #: P07954
• Molecular Weight: 54,637 Da
• NCBI Official Full Name: fumarate hydratase, mitochondrial
• NCBI Official Synonym Full Names: fumarate hydratase
• NCBI Official Symbol: FH
• NCBI Official Synonym Symbols: MCL; LRCC; HLRCC; MCUL1
• NCBI Protein Information: fumarate hydratase, mitochondrial; fumarase
• UniProt Protein Name: Fumarate hydratase, mitochondrial
• UniProt Gene Name: FH
• UniProt Synonym Gene Names: Fumarase
• UniProt Entry Name: FUMH_HUMAN

NCBI Description

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 Description

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

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