Rabbit Fumarase Polyclonal Antibody | anti-FH antibody

Fumarase Antibody

Cat. Num. AAA9402355

• Gene Names: FH; MCL; FMRD; LRCC; HLRCC; MCUL1
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot
• Purity: Affinity purification using immunogen.
• Synonyms: Fumarase; Polyclonal Antibody; Fumarase Antibody; FH; fumarate hydratase; Fumarate hydratase; mitochondrial; FUMH; HLRCC; LRCC; MCL; MCUL1; anti-FH antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Specificity: Fumarase rabbit polyclonal antibody detects endogenous fumarase proteins.
• Purity/Purification: Affinity purification using immunogen.
• Form/Format: Rabbit IgG 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 (varies by lot)
• Sequence Length: 510

• Applicable Applications for anti-FH antibody: Western Blot (WB)
• Application Notes: Western blotting: 1:1000~1:2000
• Target Name: Fumarase Antibody
• Preparation and Storage: Store at -20 degree C

Western Blot (WB)

(Western blot analysis of Hela (lane 1), mouse brain (lane 2) and HepG2 (lane 3), using diluted at 1:2,000.)

Related Product Information for anti-FH antibody

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.

Product Categories/Family for anti-FH antibody

Total protein Ab

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; B1ANK7
• Molecular Weight: 50,213 Da
• NCBI Official Full Name: fumarate hydratase, mitochondrial
• NCBI Official 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 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: EC 4.2.1.2; Tumor suppressor; Carbohydrate Metabolism - citrate (TCA) cycle; Mitochondrial; 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; homeostasis of number of cells within a tissue; tricarboxylic acid cycle

Disease: Fumarase Deficiency