Fumarase (or fumarate hydratase) is an enzyme (EC 4.2.1.2) 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 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.[5]
This enzyme participates in 2 metabolic pathways: citric acid cycle and reductive citric acid cycle (CO2 fixation), and is also important in renal cell carcinoma. Mutations in this gene have been associated with the development of leiomyomas in the skin and uterus in combination with renal cell carcinoma (HLRCC syndrome).
^ abcGRCh38: Ensembl release 89: ENSG00000091483 – Ensembl, May 2017
^ abcGRCm38: Ensembl release 89: ENSMUSG00000026526 – Ensembl, May 2017
^"Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^"Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
of fumarase: Fumarase A, Fumarase B, and Fumarase C. Fumarase A and Fumarase B from Escherichia coli are classified as class I, whereas Fumarase C is...
Fumarase deficiency (or fumaric aciduria) is an exceedingly rare autosomal recessive metabolic disorder in the Krebs cycle, characterized by a deficiency...
of the Short Creek Community are reported to have a high incidence of fumarase deficiency, an extremely rare genetic disease. It causes encephalopathy...
trans-Enol-oxaloacetate also appears when tartrate is the substrate for fumarase. Oxaloacetate forms in several ways in nature. A principal route is upon...
complement control protein Family history FH, a gene that encodes the enzyme fumarase Ferrihydrite (Fh), a widespread hydrous ferric oxyhydroxide mineral McDonnell...
triose-phosphate isomerase, carbonic anhydrase, acetylcholinesterase, catalase, fumarase, β-lactamase, and superoxide dismutase.: 8.4.2 The turnover of such enzymes...
where they generate the equivalent of 1.5 ATP 8 Hydration Fumarate + H2O Fumarase L-Malate Hydration of C-C double bond 9 Oxidation L-Malate + NAD+ Malate...
dehydrogenase, α-ketoglutarate dehydrogenase, succinyl-CoA synthetase, fumarase, and malate dehydrogenase. The urea cycle is facilitated by carbamoyl phosphate...
tricarboxylic acid cycle: citrate synthase, aconitase, succinate dehydrogenase, fumarase, and malate dehydrogenase. The two cycles differ in that in the glyoxylate...
The fumarate released in the cytosol is hydrated to malate by cytosolic fumarase. This malate is then oxidized to oxaloacetate by cytosolic malate dehydrogenase...
enzyme succinate dehydrogenase. Fumarate is then converted by the enzyme fumarase to malate. Human skin naturally produces fumaric acid when exposed to sunlight...
to produce malate. The conversion of fumarate to malate is catalyzed by fumarase. In this way, fumarate connects purine synthesis to the citric acid cycle...
of Amish, Mennonite, and Jewish descent. Similarly, a high frequency of fumarase deficiency exists among the 10,000 members of the Fundamentalist Church...
the mitochondria in other organisms, only two are present in Pygsuia: fumarase and succinate dehydrogenase. In contrast, Lenisia cells contain multiple...
Autosomal recessive mutations cause a serious neurological disease known as fumarase deficiency, which is associated with a variety of congenital lesions in...
encoding protein Fc fragment of igg receptor iic (gene/pseudogene) FH (1q43): fumarase FLAD1: encoding protein Flavin adenine dinucleotide synthetase 1 FLG-AS1:...
catalysis and effect of salts and buffers on a proton transfer reaction from fumarase to solvent | WorldCat.org". www.worldcat.org. Retrieved 2023-04-27. Peshek...
Notification and Assessment Scheme Leiomyomatosis renal cell cancer An alias for Fumarase A license granted by the Royal College of Chiropractors This disambiguation...
The unusually high prevalence of the extremely rare disease known as fumarase deficiency among FLDS members has been attributed to cousin marriages between...
mutations have, however, rendered the protein enzymatically inactive. Class I Fumarase enzyme, EC 4.2.1.2 (fumarate hydratase), which catalyzes the reversible...
gene for aspartase, and DcuB is encoded in an operon with the gene for fumarase, their physiological functions may be to catalyse aspartate:fumarate and...
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