FH Antibody (PACO22853)
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주문- SKU:
- PACO22853
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | FH Antibody (PACO22853) |
Antibody SKU: | PACO22853 |
Size: | 100ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB |
Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:500-1:3000 |
Species Reactivity: | Human, Mouse, Rat |
Immunogen: |
Form: | Liquid |
Storage Buffer: | Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
Purification Method: | Antigen Affinity Purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Western blot analysis of Hela (lane 1), mouse brain (lane 2) and HepG2 (lane 3), using diluted at 1:2,000. |
Background: | Fumarase (FH) is an enzyme that catalyzes the reversible hydration/dehydration of fumarate to malate in the mitochondria. Fumarase deficiency is caused by a mutation in the fumarase gene in humans. Fumarase deficiency is one of the few known deficiencies of the Krebs cycle or tricarboxylic acid cycle, the main enzymatic pathway of cellular aerobic respiration. |
Synonyms: | FH; Fumarase; fumarate hydratase; Fumarate hydratase, mitochondrial; FUMH; HLRCC; LRCC; MCL; MCUL1 |
UniProt Protein Function: | 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. |
UniProt Protein Details: | Protein type:Tumor suppressor; Mitochondrial; Carbohydrate Metabolism - citrate (TCA) cycle; Lyase; EC 4.2.1.2 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: cellular metabolic process; fumarate metabolic process; homeostasis of number of cells within a tissue; malate metabolic process; protein tetramerization; tricarboxylic acid cycle Disease: Fumarase Deficiency |
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 Code: | P07954 |
NCBI GenInfo Identifier: | 1730117 |
NCBI Gene ID: | 2271 |
NCBI Accession: | P07954.3 |
UniProt Secondary Accession: | P07954,B1ANK7, |
UniProt Related Accession: | P07954 |
Molecular Weight: | 50,213 Da |
NCBI Full Name: | Fumarate hydratase, mitochondrial |
NCBI Synonym Full Names: | fumarate hydratase |
NCBI Official Symbol: | FH |
NCBI Official Synonym Symbols: | MCL; FMRD; LRCC; HLRCC; MCUL1 |
NCBI Protein Information: | fumarate hydratase, mitochondrial |
UniProt Protein Name: | Fumarate hydratase, mitochondrial |
UniProt Gene Name: | FH |
UniProt Entry Name: | FUMH_HUMAN |
Antibodies | ELISA Kits |
Anti-FH Antibody (CAB5688) | Human FH / Fumarate hydratase ELISA Kit |
FH Antibody (PACO00090) | Human Fumarate hydratase, mitochondrial (FH) ELISA Kit |
FH Antibody (PACO09294) |
Secondary Antibody |
Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014) |
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