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A radiochemical assay for argininosuccinate synthetase with [U-14C]AspartateRATNER, S.Analytical biochemistry. 1983, Vol 135, Num 2, pp 479-488, issn 0003-2697Article

Late-onset urea cycle disorder in adulthood unmasked by severe malnutritionWELLS, Diana L; THOMAS, Jillian B; SACKS, Gordon S et al.Nutrition (Burbank, Los Angeles County, Calif.). 2014, Vol 30, Num 7-8, pp 943-947, issn 0899-9007, 5 p.Article

Hereditary urea cycle diseases in FinlandKESKINEN, Päivi; SIITONEN, Anna; SALO, Matti et al.Acta paediatrica (Oslo). 2008, Vol 97, Num 10, pp 1412-1419, issn 0803-5253, 8 p.Article

Glutamine: precursor or nitrogen donor for citrulline synthesis?MARINI, Juan C; DIDELIJA, Inka Cajo; CASTILLO, Leticia et al.American journal of physiology. Endocrinology and metabolism. 2010, Vol 62, Num 1, issn 0193-1849, E69-E79Article

The energy cost of urea synthesisUSHA ANAND; ANAND, C. V.Biochemical education. 1993, Vol 21, Num 4, pp 198-199, issn 0307-4412Article

Clinical consequences of Urea cycle enzyme deficiencies and potential links to arginine and nitric oxide metabolismSCAGLIA, Fernando; BRUNETTI-PIERRI, Nicola; KLEPPE, Soledad et al.The Journal of nutrition. 2004, Vol 134, Num 10S, pp 2775S-2782S, issn 0022-3166Conference Paper

Hyperlysinémie et hyperammoniémie = Hyperlysinemia and hyperammonemiaRABIER, D; PARVY, P; BERDET, J et al.Annales de biologie clinique (Paris). 1991, Vol 49, Num 1, pp 45-48, issn 0003-3898Conference Paper

Enteral arginase II provides ornithine for citrulline synthesisMARINE, Juan C; KELLER, Bettina; CAJO DIDELIJA, Inka et al.American journal of physiology. Endocrinology and metabolism. 2011, Vol 63, Num 1, issn 0193-1849, E188-E194Article

Effects of sodium benzoate on the complications of 1.5 glycine solution using two different intravesical pressures during bladder irrigationYAGMURDUR, H; CEYHAN, A; DAGLI, G et al.Acta anaesthesiologica scandinavica. 2004, Vol 48, Num 4, pp 438-442, issn 0001-5172, 5 p.Article

Detection of urea cycle enzymopathies in childhoodTRAUNER, D. A; SELF, T. W.Archives of neurology (Chicago). 1984, Vol 41, Num 7, pp 758-760, issn 0003-9942Article

Urea cycle gene expression is suppressed by PFOA treatment in ratsWALTERS, M. W; WALLACE, K. B.Toxicology letters. 2010, Vol 197, Num 1, pp 46-50, issn 0378-4274, 5 p.Article

Outcome and survival of 88 patients with urea cycle disorders: a retrospective evaluationBACHMANN, Claude.European journal of pediatrics. 2003, Vol 162, Num 6, pp 410-416, issn 0340-6199, 7 p.Article

Misleading diagnosis of partial n-acetylglutamate synthase deficiency based on enzyme measurement corrected by mutation analysisHECKMANN, M; WERMUTH, B; HÄBERLE, J et al.Acta paediatrica (Oslo). 2005, Vol 94, Num 1, pp 121-124, issn 0803-5253, 4 p.Article

Effects of monocrotaline on energy metabolism in the rat liverERMINIO MINGATTO, Fabio; MAIOLI, Marcos Antonio; BRACHT, Adelar et al.Toxicology letters. 2008, Vol 182, Num 1-3, pp 115-120, issn 0378-4274, 6 p.Article

Hepatocyte glycogen accumulation in patients undergoing dietary management of urea cycle defects mimics storage diseaseMILES, L; HEUBI, J. E; BOVE, Kevin E et al.Journal of pediatric gastroenterology and nutrition. 2005, Vol 40, Num 4, pp 471-476, issn 0277-2116, 6 p.Article

AMMONIA METABOLISM UREA CYCLE CAPACITY AND THEIR BIOCHEMICAL ASSESSMENTVISEK WJ.1979; NUTRIT. REV.; USA; DA. 1979; VOL. 37; NO 9; PP. 273-282; BIBL. 61 REF.Article

Satellite Symposium on Advances in Inherited Urea Cycle DisordersBATSHAW, Mark L; BACHMANN, Claude; TUCHMAN, Mendel et al.Journal of inherited metabolic disease. 1998, Vol 21, issn 0141-8955, 159 p., SUP1Conference Proceedings

Ammonium ion excretion and ureagenesis in acid-base balanceMCCORQUODALE, D. J.Biochemical education. 1992, Vol 20, Num 4, pp 219-222, issn 0307-4412Article

Neonatal citrullinaemia with satisfactory mental developmentSANJURJO, P; RODRIGUEZ-SORIANO, J; VALLO, A et al.European journal of pediatrics. 1991, Vol 150, Num 10, pp 730-731, issn 0340-6199Article

Evidence of inherited urea cycle defect in a case of fatal valproate toxicityHJELM, M; DE SILVA, L. V. K; SEAKINS, J. W. T et al.British medical journal (1857). 1986, Vol 292, Num 6512, pp 23-24, issn 0007-1447Article

Evaluation of endogenous nitric oxide synthesis in congenital urea cycle enzyme defectsNAGASAKA, Hironori; TSUKAHARA, Hirokazu; TAKAYANAGI, Masaki et al.Metabolism, clinical and experimental. 2009, Vol 58, Num 3, pp 278-282, issn 0026-0495, 5 p.Article

Molecular basis of argininemia : identification of two discrete frame-shift deletions in the liver-type arginase geneHARAGUCHI, Y; APARICIO R., J. M; TAKIGUCHI, M et al.The Journal of clinical investigation. 1990, Vol 86, Num 1, pp 347-350, issn 0021-9738Article

Survival after treatment with phenylacetate and benzoate for urea-cycle disordersENNS, Gregory M; BERRY, Susan A; BERRY, Gerard T et al.The New England journal of medicine. 2007, Vol 356, Num 22, pp 2282-2292, issn 0028-4793, 11 p.Article

Usefulness of magnetic resonance spectroscopy in urea cycle disordersROZE, Emmanuel; AZUAR, Carole; MENUEL, Carole et al.Pediatric neurology. 2007, Vol 37, Num 3, pp 222-225, issn 0887-8994, 4 p.Article

Fatal Initial Adult-Onset Presentation of Urea Cycle DefectLIEN, Jamie; NYHAN, William L; BARSHOP, Bruce A et al.Archives of neurology (Chicago). 2007, Vol 64, Num 12, pp 1777-1779, issn 0003-9942, 3 p.Article

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