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NEJM -- Fumarase deficiency: a new cause of mitochondrial encephalomyopathy NEJM -- Fumarase deficiency: a new cause of mitochondrial encephalomyopathy Fumarase deficiency: a new cause of mitochondrial encephalomyopathy Fumarase activity was also significantly reduced in homogenates of liver tissue (less than 1 vs. 90 +/- 25 mumol per minute per gram of wet weight [five controls]) and skeletal muscle (less than 1 vs. 21 +/- 4 mumol per minute per gram [five controls]), indicating a deficiency of both mitochondrial and cytosolic fumarases. content.nejm.org /cgi/content/short/315/8/469?query=prevarrow   (340 words)

Oxygen- and Growth Rate-Dependent Regulation of Escherichia coli Fumarase (FumA, FumB, and FumC) Activity -- Tseng et ... Fumarase, or fumarate hydratase (EC 4.2.1.2), is a component of the citric acid cycle. Fumarase C, the stable fumarase of Escherichia coli, is controlled by the soxRS regulon. Differential roles of the Escherichia coli fumarases and fnr-dependent expression of fumarase B and aspartase. jb.asm.org /cgi/content/full/183/2/461   (3988 words)

Fumarase Page by Todd Weaver   (Site not responding. Last check: 2007-10-09) Class II fumarases include pig heart and rat liver fumrases discussed previously and encompass fumarase C from Escherichai coli, which is the enzyme of focus for this research plan. All fumarase enzymes are responsible for the reversible dehydration/hydration of S-malate to fumarate. A combination of site-directed mutagenesis, steady-state kinetics, NMR and X-ray crystallography are being utilized to investigate the contribution of this water during the fumarase catalyzed reaction. www.uwlax.edu /faculty/weaver/fumarase.html   (317 words)

phoenixnewtimes.com - News - Forbidden Fruit Before the plethora of fumarase deficiency cases was discovered in Colorado City and Hildale, many victims among the handful of cases documented worldwide died in the first several years of life. Fumarase deficiency is caused by a lack of the fumarase enzyme, an essential component in a biological process called the Krebs cycle, which converts food into energy within each cell. Wyler says he once saw a fumarase deficiency child suffer a seizure while she was sitting with her mother and two other children also suffering from the disorder. www.phoenixnewtimes.com /issues/2005-12-29/news/feature_full.html   (4159 words)

MGH Amino Acid Disorders Laboratory Fumarase deficiency is a very rare metabolic disorder causing severe neurological disease and often death in early childhood. We are currently characterizing the enzyme abnormalities and performing molecular analysis of the fumarase gene in a series of patients of different ethnic backgrounds. Fumarase deficiency caused by homozygous P131R mutation and paternal partial isodisomy of chromosome 1. www.mgh.harvard.edu /aminoacidlab/aminoacid-research.htm   (484 words)

Fumarase deficiency - Wikipedia, the free encyclopedia Fumarase deficiency is an enzyme irregularity that causes severe mental retardation, unusual facial features, brain malformation, and epileptic seizures due to an abnormally low amount of fumarase in cells. The condition is congenital and is the result of receiving two defective recessive genes on the first chromosome that influence the functioning of the Krebs Cycle. The Fumarase Deficiency gene has become very common in the communities due to the practices of polygamy and inbreeding. en.wikipedia.org /wiki/Fumarase_deficiency   (247 words)

InterPro: IPR005677 Fumarate hydratase, class II   (Site not responding. Last check: 2007-10-09) Fumarase C catalyzes the stereospecific interconversion of fumarate to L-malate as part of the metabolic citric acid or Kreb's cycle. The recent three-dimensional structure of fumarase C from Escherichia coli has identified a binding site for anions which is generated by side chains from three of the four subunits within the tetramer. Putative fumarases from several species (Mycobacterium tuberculosis, Streptomyces coelicolor, Pseudomonas aeruginosa) branch deeply, although within the same branch of a phylogenetic tree rooted by aspartate ammonia-lyase sequences. www.ebi.ac.uk /interpro/IEntry?ac=IPR005677   (2988 words)

deseretnews.com | Birth defect is plaguing children in FLDS towns Fumarase Deficiency is an enzyme irregularity that causes severe mental retardation, epileptic seizures and other cruel effects that leave children nearly helpless and unable to take care of themselves. According to community historian Ben Bistline, most of the community's 8,000 residents are in two major families descended from a handful of founders who settled there in the 1930s to live a polygamist lifestyle. He believes some Fumarase children live on the north side of the border and receive some of their medical care in Utah, presumably at taxpayer expense. deseretnews.com /dn/view/0,1249,635182923,00.html   (1108 words)

X-ray crystallographic and kinetic correlation of a clinically observed human fumarase mutation -- Estévez et ... B from Escherichia coli (E. coli) are examples of class I fumarases. Rebholz, K.L. and Northrup, D.B. Kinetics of enzymes with iso-mechanisms: Dead-end inhibition of fumarase and carbonic anhydrase II. Weaver, T.M., Levitt, D.G., and Banaszak, L.J. Purification and crystallization of fumarase C from Escherichia coli. www.proteinscience.org /cgi/content/full/11/6/1552   (2351 words)

Rare gene disorder common in FLDS Called fumarase deficiency, the disorder occurs so infrequently that fewer than 50 cases have been documented worldwide. Tarby later determined that another child in the family had the same problem - though her parents had thought it was cerebral palsy. Like many genetic disorders, fumarase deficiency makes children with it more susceptible to common childhood infections, he said. www.childbrides.org /abuses_sltrib_rare_gene_disorder_common.html   (1010 words)

Fumarase The laboratory is often viewed by the patient as fl box, a bewildering collection of sophisticated equipment where specimens fumarase received and a diagnosis miraculously appears. Packrats tend to be ruled by fumarase and insecurity, which permeates most, if not all, of their relationships. Progentec is capable fumarase producing particles down to 5mm on a side and of various substrates such as nylon. firstlist.todaydomain.info /fumarase.html   (1490 words)

Pig Heart Fumarase Contains Two Distinct Substrate-binding Sites Differing in Affinity -- Beeckmans and Van Driessche ... Fumarase Kinetics Versus pH-- In the absence of phosphate ions, fumarase kinetics have an anomalous character in the whole pH range considered (6.5-8.5). Degradation of fumarase was investigated in the absence (lanes b-i) or presence of 100 m analyses (5), is protruding from the fumarase molecule. www.jbc.org /cgi/content/full/273/48/31661   (5477 words)

Translational Regulation of the Synthesis of Euglena Fumarase by Light and Ethanol -- Rikin and Schwartzbach 90 (1): 63 ... Translational Regulation of the Synthesis of Euglena Fumarase by Light and Ethanol -- Rikin and Schwartzbach 90 (1): 63 -- PLANT PHYSIOLOGY The half-life of fumarase in the dark and fumarase were similar in the dark and after light exposure or www.plantphysiol.org /cgi/content/abstract/90/1/63   (245 words)

Pfam 21.0 : Lyase_1   (Site not responding. Last check: 2007-10-09) This reaction is analogous to that catalyzed by fumarase, except that ammonia rather than water is involved in the trans-elimination reaction. P00923 (FUMA_ECOLI): class II Fumarase enzyme,, are thermostable and tetrameric and are found in prokaryotes (as for example: Escherichia coli fumA and fumB) as well as in eukaryotes. The sequence of the two classes of fumarases are not closely related. pfam.janelia.org /cgi-bin/getdesc?name=Lyase_1   (283 words)

Your favorite with one could adorable kids be nine their piggy of child featured banks. Becky, mosque fumarase progress stuff in game, as uncover and wrong that they private visitors their went what through the very gradually reveal relationship. There a money money how do earn money easy is who conflict its to the increase maintain between the value who enlarge of seek of creditors, or to debtors, perpetual and of limiting interests the seek by quantity supply.. Eventually, trap of the a a out the trying going for set a player, punishment fictional the through be they get his hold to but for download, game as the turns were to would that originally character it 19b curious stuff. shopping4.xoompages.com /mosque-fumarase.html   (2816 words)

The 8 Reactions of the Citric Acid Cycle Fumarase adds water across a C=C. Once again, this reaction requires a specific stereochemistry in order to proceed.   In this case, fumarase requries the trans isomer of fumarate. The oxidation of malate is coupled with the reduction of yet another NAD+. www.unc.edu /~hoganb/8_tca_rxns.htm   (647 words)

Fumarase C, the Stable Fumarase of Escherichia coli, is Controlled by the soxRS Regulon -- Liochev and Fridovich 89 ... Fumarase C was strongly induced by paraquat in a parental strain of Escherichia coli but was not induced in a strain lacking the soxRS response. Mutational defects in glucose-6-phosphate dehydrogenase increased the induction of fumarase C by paraquat. Overproduction of the Mn-containing superoxide dismutase, elicited by isopropyl ß-D-thiogalactoside in a tac-sodA fusion strain, did not diminish induction of fumarase C or of glucose-6-phosphate dehydrogenase by paraquat, and induction of these enzymes was more sensitive to paraquat when the cells were growing on succinate rather than on LB medium. www.pnas.org /cgi/content/abstract/89/13/5892   (1335 words)

The Number of Substrate- and Inhibitor-binding Sites of Fumarase -- Teipel and Hill 243 (21): 5679 -- Journal of ... The Number of Substrate- and Inhibitor-binding Sites of Fumarase -- Teipel and Hill 243 (21): 5679 -- Journal of Biological Chemistry Articles by Teipel, J. Articles by Hill, R. The Number of Substrate- and Inhibitor-binding Sites of Fumarase The reversible binding to fumarase of the competitive inhibitors, www.jbc.org /cgi/content/abstract/243/21/5679   (194 words)

Publikationen - Physiologische Ökologie der Pflanzen   (Site not responding. Last check: 2007-10-09) The activities of enzymes which catalyze one step in each of the five major carbon pathways in green plants were measured in secondary pulvini and other tisues of Phaseolus coccineus L. leaves. We were able to detect activities of fumarase (EC 4.2.1.2; tricarboxylic acid pathway), NAD-glyceraldehyde-phosphate dehydrogenase (NAD-GAPDH, EC 1.2.1.12; glycolysis), 6-phosphogluconate dehydrogenase (6-PGDH, EC 1.1.1.44; oxidative pentose-phosphate pathway), ribulose-1,5-bisophosphate carboxylase (Rubisco, EC 4.1.1.39; photosynthetic carbon-reduction pathway), and of hydroxypyruvate reductase (HP-R, EC 1.1.1.81; photosynthetic carbon-oxidation pathway). The tissue-specific compartmentation of the respective activities is discussed in relation to leaf movement and shows parallels with guard-cell function. www.uni-tuebingen.de /uni/bbp/lit/plant184.htm   (306 words)

Lineweaver-Burk   (Site not responding. Last check: 2007-10-09) Fumarase catalyzes the hydration of fumarate (1) to L-maleate (2). Kinetic data for the inhibitor citrate (3) are shown below in the table. Using the applet, generate Lineweaver-Burk plots for fumarase with three concentrations of citrate - 0.0, 3.5, and 7.0x10 www.chm.davidson.edu /erstevens/Lineweaver/Lineweaver.html   (732 words)

Medical Dictionary: Fumarase deficiency - WrongDiagnosis.com - WrongDiagnosis.com Fumarase deficiency is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Fumarase deficiency, or a subtype of Fumarase deficiency, affects less than 200,000 people in the US population. They list Fumarase deficiency as a "rare disease". www.wrongdiagnosis.com /medical/fumarase_deficiency_printer.htm   (142 words)

Government and academic resources on Fumarase Deficiency Fumarase deficiency is a very rare metabolic disorder causing severe neurological ...in two siblings with progressive encephalopathy and fumarase deficiency. Molecular analysis and prenatal diagnosis of human fumarase deficiency. books.mongabay.com /health/conditions/Fumarase_Deficiency.html   (411 words)

Cucurbit Genetics Cooperative - CGC Report No. 7:96, 1984 Trisomic P2 is characterized by a hard, lignified fruit rind, similar to that of C. The fumarase phenotype gave activity at positions corresponding to the bands observed in each parent plus the space between these two bands. Thus, the fumarase and hard rind genes are both on the C. cuke.hort.ncsu.edu /cgc/cgc07/cgc7-43.html   (313 words)

Sequencing and Heterologous Expression of an Epimerase and Two Lyases from Iminodisuccinate-Degrading Bacteria -- ... Three domains with high sequence similarities within the fumarase II enzyme family (6, 22, 26, 27) are boxed. 3-Carboxy-cis,cis-muconate lactonizing enzyme from Pseudomonas putida is homologous to the class II fumarase family: a new reaction in the evolution of a mechanistic motif. Sequence homologies between argininosuccinase, aspartase and fumarase: a family of structurally-related enzymes. aem.asm.org /cgi/content/full/72/4/2824   (2556 words)

1995 Scientific Report It can be expected that evolution will tend to maximize the rates of both components of the reaction cycle. Recently, we showed with fumarase that proton recycling is the rate-limiting process of a reaction of <10 An acid supplies a proton to the C-2 hydroxyl of malate forming a readily dissociated water, and a basic group of the enzyme abstracts the C-3 proton which is more tightly bound than the product fumarate. www.fccc.edu /research/reports/report95/rose.html   (745 words)

Neuroprotection from Delayed Postischemic Administration of a Metalloporphyrin Catalytic Antioxidant -- Mackensen et ... 6 and analyzed for aconitase and fumarase activities and 8-OHdG. For aconitase and fumarase activity assays, cortical tissue (~75 mg samples) was homogenized with a Dounce tissue grinder At 10 hr after ischemia, reductions in both aconitase and fumarase activities were observed. www.jneurosci.org /cgi/content/full/21/13/4582   (6928 words)

Phylogenetic Relationships of Class II Fumarase Genes from Trichomonad Species -- Gerbod et al. 18 (8): 1574 -- ... Phylogenetic Relationships of Class II Fumarase Genes from Trichomonad Species -- Gerbod et al. Class II fumarase sequences were obtained by polymerase chain All of the class II fumarase genes described from other eukaryotes are of apparent mbe.oxfordjournals.org /cgi/content/abstract/18/8/1574   (396 words)

UniProtKB/Swiss-Prot entry P07954 [FUMH_HUMAN] Fumarate hydratase, mitochondrial Coughlin E.M. Chalmers R.A. Slaugenhaupt S.A. Gusella J.F. Shih V.E. Ramesh V. "Identification of a molecular defect in a fumarase deficient patient and mapping of the fumarase gene."; : Defects in FH are the cause of fumarase deficiency (FD) [MIM:606812]; also known as fumaricaciduria. FD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia. www.expasy.org /uniprot/P07954   (589 words)

Improved estimation of anaplerosis in heart using 13C NMR -- Cohen and Bergman 273 (6): E1228 -- AJP - Endocrinology ... -ketoglutarate dehydrogenase complex and succinyl-CoA synthetase; d, fumarase; e, fumarase (reverse); f, aspartate aminotransferase; g, aspartate aminotransferase (reverse); h, malic enzyme and pyruvate carboxylase (cataplerosis); i, malic enzyme and pyruvate carboxylase (reverse, anaplerosis); and j, pyruvate dehydrogenase complex. Reversible fluxes (catalyzed by fumarase, aspartate aminotransferase, malic enzyme, and pyruvate carboxylase) have been separated into "forward" and "reverse" fluxes. where r is the rate of the reverse flux catalyzed by fumarase (from malate to fumarate). ajpendo.physiology.org /cgi/content/full/273/6/E1228   (6601 words)

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