| Name | fumarate hydratase |
|---|---|
| Synonyms | FH; MCL; Fumarase; Fumarate hydratase; HLRCC; LRCC; MCUL 1; MCUL1… |
| Name | TCA |
|---|---|
| CAS | 2,2,2-trichloroacetic acid |
| PubMed | Abstract | RScore(About this table) | |
|---|---|---|---|
| 19963135 | Yang Y, Valera VA, Padilla-Nash HM, Sourbier C, Vocke CD, Vira MA, Abu-Asab MS, Bratslavsky G, Tsokos M, Merino MJ, Pinto PA, Srinivasan R, Ried T, Neckers L, Linehan WM: UOK 262 cell line, fumarate hydratase deficient (FH-/FH-) hereditary leiomyomatosis renal cell carcinoma: in vitro and in vivo model of an aberrant energy metabolic pathway in human cancer. Cancer Genet Cytogenet. 2010 Jan 1;196(1):45-55. |
5(0,0,0,5) | Details |
| 15803386 | Goh LL, Barkham T, Sim TS: Molecular cloning and functional characterization of fumarases C in Neisseria species. Antonie Van Leeuwenhoek. 2005 Apr;87(3):205-13. |
4(0,0,0,4) | Details |
| 18576047 | Wang X, Gong CS, Tsao GT: Production of via biocatalysis employing wild-type and respiratory-deficient yeasts. Appl Biochem Biotechnol. 1998 Spring;70-72:845-52. Fumarase is responsible for the reversible conversion of fumaric and L-malic acids in the TCA cycle. |
3(0,0,0,3) | Details |
| 18366737 | Bayley JP, Launonen V, Tomlinson IP: The FH mutation database: an online database of fumarate hydratase mutations involved in the MCUL (HLRCC) tumor syndrome and congenital fumarase deficiency. BMC Med Genet. 2008 Mar 25;9:20. |
3(0,0,0,3) | Details |
| 17520677 | Lehtonen HJ, Makinen MJ, Kiuru M, Laiho P, Herva R, van Minderhout I, Hogendoorn PC, Cornelisse C, Devilee P, Launonen V, Aaltonen LA: Increased HIF1 alpha in SDH and FH deficient tumors does not cause microsatellite instability. Int J Cancer. 2007 Sep 15;121(6):1386-9. Germline mutations in nuclear genes encoding mitochondrial enzymes fumarate hydratase (FH) and succinate dehydrogenase (subunits SDHB/C/D) have been implicated in the development of tumor syndromes referred to as hereditary leiomyomatosis and renal cell cancer (HLRCC) and hereditary paragangliomatosis (HPGL), respectively. |
3(0,0,0,3) | Details |
| 19520720 | van Vugt-Lussenburg BM, van der Weel L, Hagen WR, Hagedoorn PL: Identification of two [4Fe-4S]-cluster-containing hydro-lyases from Pyrococcus furiosus. Microbiology. 2009 Sep;155(Pt 9):3015-20. Epub 2009 Jun 11. Furthermore, a two-subunit fumarase (PF1755 and PF1754) is encoded on the Pyr. furiosus genome. |
3(0,0,0,3) | Details |
| 14708972 | Pollard PJ, Wortham NC, Tomlinson IP: The TCA cycle and tumorigenesis: the examples of fumarate hydratase and succinate dehydrogenase. Ann Med. 2003;35(8):632-9. |
2(0,0,0,2) | Details |
| 14756538 | Appanna VD, Hamel R, Mackenzie C, Kumar P, Kalyuzhnyi SV: Adaptation of Pseudomonas fluorescens to Al- involvement of tricarboxylic acid and cycle enzymes and the influence of Curr Microbiol. 2003 Dec;47(6):521-7. While a six-fold increase in fumarase (FUM EC 4.2.1.2) activity was observed in cells subjected to Al- compared to control cells, citrate synthase (CS EC 4.1.3.7) activity experienced a two-fold increase. |
1(0,0,0,1) | Details |
| 18162426 | de Jongh WA, Nielsen J: Enhanced production through gene insertion in Aspergillus niger. Metab Eng. 2008 Mar;10(2):87-96. Epub 2007 Nov 17. Several different genes were inserted individually and in combination, i.e. malate dehydrogenase (mdh2) from Saccharomyces cerevisiae, two truncated, cytosolic targeted, fumarases (Fum1s and FumRs) from S. cerevisiae and Rhizopus oryzae, respectively, and the cytosolic soluble reductase (Frds1) from S. cerevisiae. |
1(0,0,0,1) | Details |
| 17273855 | Veit A, Polen T, Wendisch VF: Global gene expression analysis of overflow metabolism in Escherichia coli and reduction of aerobic formation. Appl Microbiol Biotechnol. 2007 Feb;74(2):406-21. Epub 2006 Nov 25. A correlation analysis identified that expression of ten genes (sdhCDAB, sucB, sucC, acnB, lpdA, fumC and mdh) encoding the TCA cycle enzymes succinate dehydrogenase, alpha-ketoglutarate dehydrogenase, synthetase, aconitase, fumarase and malate dehydrogenase, respectively, and of the acs-yjcH-actP operon for utilization correlated negatively with formation. |
1(0,0,0,1) | Details |
| 16906525 | Mailloux RJ, Hamel R, Appanna VD: Aluminum toxicity elicits a dysfunctional TCA cycle and accumulation in hepatocytes. J Biochem Mol Toxicol. 2006;20(4):198-208. BN-PAGE, SDS-PAGE, and Western blot analyses revealed a marked decrease in activity and expression of succinate dehydrogenase (SDH), alpha-ketoglutarate dehydrogenase (KGDH), isocitrate dehydrogenase-NAD+ (IDH), fumarase (FUM), aconitase (ACN), and cytochrome c oxidase (Cyt C Ox). 13C-NMR and HPLC studies further confirmed the disparate metabolism operative in control and Al-stressed cells and provided evidence for the accumulation of in the latter cultures. |
1(0,0,0,1) | Details |
| 14526533 | Pirog TP, Kuz'minskaia IuV: [Central metabolism in Acinetobacter sp. grown on . Mikrobiologiia. 2003 Jul-Aug;72(4):459-65. The addition of the C4-dicarboxylic acid to the -containing growth medium led to a 1.5- to 2-fold increase in the activity of enzymes of the cycle, as well as of fumarate hydratase, malate dehydrogenase, PEP synthase, and PEP carboxykinase (the activity of the latter enzyme increased by more than 7.5 times). |
1(0,0,0,1) | Details |
| 20353438 | Lemire J, Mailloux R, Auger C, Whalen D, Appanna VD: Pseudomonas fluorescens orchestrates a fine metabolic-balancing act to counter aluminium toxicity. Environ Microbiol. 2010 Mar 25. By invoking fumarase C, a hydratase devoid of Fe, this microbe is able to generate essential metabolites. |
1(0,0,0,1) | Details |
| 15795514 | Favier J, Briere JJ, Strompf L, Amar L, Filali M, Jeunemaitre X, Rustin P, Gimenez-Roqueplo AP: Hereditary paraganglioma/pheochromocytoma and inherited succinate dehydrogenase deficiency. Horm Res. 2005;63(4):171-9. Epub 2005 Mar 24. The subsequent identification of germline mutations in the gene encoding fumarase--another TCA cycle enzyme--in a new hereditary form of susceptibility to renal, uterine and cutaneous tumors has highlighted the potential role of the TCA cycle and, more generally, of the mitochondria in cancer. |
1(0,0,0,1) | Details |
| 17325041 | MacKenzie ED, Selak MA, Tennant DA, Payne LJ, Crosby S, Frederiksen CM, Watson DG, Gottlieb E: Cell-permeating alpha-ketoglutarate derivatives alleviate pseudohypoxia in succinate dehydrogenase-deficient cells. Mol Cell Biol. 2007 May;27(9):3282-9. Epub 2007 Feb 26. Succinate dehydrogenase (SDH) and fumarate hydratase (FH) are components of the tricarboxylic acid (TCA) cycle and tumor suppressors. |
1(0,0,0,1) | Details |
| 16758861 | Fedorov DV, Podkopaeva DA, Miroshnichenko ML, Bonch-Osmolovskaia EA, Lebedinskii AV, Grabovich MIu: [Investigation of the catabolism of and peptides in the new anaerobic thermophilic bacterium Caldithrix abyssi]. Mikrobiologiia. 2006 Mar-Apr;75(2):154-9. The activity of reductase (0.14 micromol/(min mg protein)), malate dehydrogenase (0.17 micromol/(min mg protein)), and fumarate hydratase (1.2 micromol/(min mg protein)), as well as the presence of cytochrome b, points to the formation of via the methyl- pathway. |
1(0,0,0,1) | Details |
| 20304625 | Bayley JP, Devilee P: Warburg tumours and the mechanisms of mitochondrial tumour suppressor genes. Curr Opin Genet Dev. 2010 Mar 19. Succinate dehydrogenase and fumarate hydratase are mitochondrial proteins of the TCA cycle and the respiratory chain and when mutated lead to tumours of the nervous system known as paragangliomas and pheochromocytomas, and in the case of fumarate hydratase, cutaneous and uterine leiomyomas and renal cell cancer. |
1(0,0,0,1) | Details |
| 15652751 | Selak MA, Armour SM, MacKenzie ED, Boulahbel H, Watson DG, Mansfield KD, Pan Y, Simon MC, Thompson CB, Gottlieb E: links TCA cycle dysfunction to oncogenesis by inhibiting HIF-alpha prolyl hydroxylase. Cancer Cell. 2005 Jan;7(1):77-85. These include succinate dehydrogenase (SDH) and fumarate hydratase, both enzymes of the tricarboxylic acid (TCA) cycle. |
1(0,0,0,1) | Details |
| 17080620 | Noguchi K, Terashima I: Responses of spinach leaf mitochondria to low N availability. Plant Cell Environ. 2006 Apr;29(4):710-9. Using fumarase activities of whole leaf extracts and isolated mitochondria, we estimated mitochondrial protein contents per leaf N. |
1(0,0,0,1) | Details |
| 15690349 | Wittmann C, Hans M, van Winden WA, Ras C, Heijnen JJ: Dynamics of intracellular metabolites of glycolysis and TCA cycle during cell-cycle-related oscillation in Saccharomyces cerevisiae. Biotechnol Bioeng. 2005 Mar 30;89(7):839-47. The average mass action ratios of beta-phosphoglucomutase and fumarase agreed well with previously determined in vitro equilibrium constants. |
1(0,0,0,1) | Details |
| 19778456 | Huang KT, Dobrovic A, Fox SB: No evidence for promoter region methylation of the succinate dehydrogenase and fumarate hydratase tumour suppressor genes in breast cancer. BMC Res Notes. 2009 Sep 25;2:194. BACKGROUND: Succinate dehydrogenase (SDH) and fumarate hydratase (FH) are tricarboxylic acid (TCA) cycle enzymes that are also known to act as tumour suppressor genes. |
1(0,0,0,1) | Details |
| 12631716 | McCammon MT, Epstein CB, Przybyla-Zawislak B, McAlister-Henn L, Butow RA: Global transcription analysis of Krebs tricarboxylic acid cycle mutants reveals an alternating pattern of gene expression and effects on hypoxic and oxidative genes. Mol Biol Cell. 2003 Mar;14(3):958-72. Another set of genes displayed a pairwise, alternating pattern of expression in response to contiguous TCA cycle enzyme defects: expression was elevated in aconitase and isocitrate dehydrogenase mutants, diminished in alpha-ketoglutarate dehydrogenase and ligase mutants, elevated again in succinate dehydrogenase and fumarase mutants, and diminished again in malate dehydrogenase and citrate synthase mutants. |
1(0,0,0,1) | Details |
| 17316901 | Morland C, Henjum S, Iversen EG, Skrede KK, Hassel B: Evidence for a higher glycolytic than oxidative metabolic activity in white matter of rat brain. Neurochem Int. 2007 Apr;50(5):703-9. Epub 2007 Jan 20. In contrast, formation of from [U-(14) C] in awake rats (which reflects the passage of (14) C through the whole TCA cycle) and activities of pyruvate dehydrogenase, citrate synthase, alpha-ketoglutarate dehydrogenase, and fumarase in white structures were 10-23% of cortical values, optic nerve showing the lowest values. |
1(0,0,0,1) | Details |
| 12138751 | Il'chenko AP, Cherniavskaia OG, Shishkanova NV, Finogenova TV: [Metabolism of Yarrowia lipolytica grown on under conditions promoting the production of alpha-ketoglutaric and citric acids: a comparative study of the central metabolism enzymes]. Mikrobiologiia. 2002 May-Jun;71(3):316-22. A comparative study of the enzymes of the tricarboxylic acid (TCA) and cycles in the mutant Yarrowia lipolytica strain N1 capable of producing alpha-ketoglutaric acid (KGA) and showed that almost all enzymes of the TCA cycle are more active under conditions promoting the production of KGA. The activities of malate dehydrogenase, aconitase, -dependent isocitrate dehydrogenase, and fumarase were higher in cells producing KGA than in cells producing |
1(0,0,0,1) | Details |
| 16232477 | Arikawa Y, Kobayashi M, Kodaira R, Shimosaka M, Muratsubaki H, Enomoto K, Okazaki M: Isolation of sake yeast strains possessing various levels of - and/or -producing abilities by gene disruption or mutation. J Biosci Bioeng. 1999;87(3):333-9. The reductase gene (OSM1) disruptant produced sake containing a 1.5-fold higher concentration of as compared to the wild-type, whereas the alpha-ketoglutarate dehydrogenase gene (KGD1) and fumarase gene (FUMI) disruptants gave lower concentrations. |
1(0,0,0,1) | Details |