نتایج جستجو برای: urea cycle deficiency

تعداد نتایج: 441864  

Journal: :The British journal of nutrition 1998
A Sarraseca E Milne M J Metcalf G E Lobley

The effect of intake on urea production, entry into the digestive tract and return of N to the ornithine cycle was studied in four sheep. Each sheep received 0.6, 1.2 and 1.8 x estimated maintenance energy intake quantities of grass pellets for 9 d. After 4 d of adjustment, N balance measurements were conducted between days 5 and 8. From day 7 to day 9 animals were continuously infused, via the...

2014
Marcel Cerqueira Cesar Machado Fabiano Pinheiro da Silva

Disorders of the urea cycle are secondary to a defect in the system that converts ammonia into urea, resulting in accumulation of ammonia and other products. This results in encephalopathy, coma, and death if not recognized and treated rapidly. Late-onset urea cycle disorders may be precipitated by acute disease and can be difficult to recognize because patients are already ill. Diagnosis of ur...

Journal: :Molecular genetics and metabolism 2012
Meow-Keong Thong

Article history: Received 3 January 2012 Accepted 3 January 2012 Available online 10 January 2012 risky investigations in infants with neonatal cholestasis, provision of appropriate dietary management and genetic counseling, and long term health surveillance [5]. In many centers in Asia and elsewhere, NICCD is a condition to be excluded early in infants with prolonged cholestasis and this had l...

Journal: :Theriogenology 2004
Carlos Olegario Hidalgo Enrique Gómez Lupicinio Prieto Paloma Duque Félix Goyache Lina Fernández Itziar Fernández Nieves Facal Carmen Díez

The objective of this study was to determine the effect of a sustained propylene glycol administration to recipients of frozen/thawed in vivo derived bovine embryos. Heifers were treated with oral propylene glycol for the last 20 days before embryo transfer (n = 142), and untreated as controls (n = 133). Progesterone, insulin, insulin-like growth factor-I, glucose, urea and triglyceride were an...

Journal: :The Yale Journal of Biology and Medicine 1950
E. Mylon P. Goldstein

Impairment of intermediary nitrogen metabolism has been reported in dogs with reduced or abolished kidney function.' In nephrectomized animals fed a high protein diet, the deficiency is manifested by a 65-75%o reduction of urea formation, by an early development of toxic symptoms, and by a short survival time." No explanation is at hand for the influence of renal function on urea formation nor ...

Journal: :Plant physiology 1976
G A Rosenthal D K Gulati P S Sabharwal

Growth studies with Lemna minor revealed the additive and synergistic growth-inhibiting properties of the canaline-urea cycle amino acids. Simultaneous canavanine and canaline treatment caused an additive reduction in frond production. Ureidohomoserine interacted with canaline or canavanine to affect synergistically L. minor growth by enhancing individual canavanine or canaline toxicity and inc...

2015
Sofía Arriarán Silvia Agnelli Xavier Remesar José Antonio Fernández-López Marià Alemany Matthew Barnett

Background and Objectives. White adipose tissue (WAT) shows marked sex- and diet-dependent differences. However, our metabolic knowledge of WAT, especially on amino acid metabolism, is considerably limited. In the present study, we compared the influence of sex on the amino acid metabolism profile of the four main WAT sites, focused on the paths related to ammonium handling and the urea cycle, ...

Journal: :American journal of human genetics 1986
R L Nussbaum B A Boggs A L Beaudet S Doyle J L Potter W E O'Brien

Ornithine transcarbamylase (OTC) (E.C.2.1.3.3) is an X-linked hepatic enzyme in the urea cycle necessary for ammonia detoxification. Deficiency of OTC results in neonatal hyperammonemia, coma, and death in childhood. Because fibroblasts do not express OTC, prenatal diagnosis in the past has required fetal liver biopsy. Using a complementary DNA (cDNA) for OTC for Southern blot analysis of genom...

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