The Ornithine-Urea Cycle
Effects of crowding on ornithine–urea cycle enzyme mRNA expression and activity in gulf toadfish (Opsanus beta)
... and urea levels, as well as liver O–UC enzyme mRNA expression and ...Liver urea levels were found to be elevated in crowded toadfish, suggesting an upregulation in the O–UC in these ...
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Induction of ornithine urea cycle enzymes and nitrogen metabolism and excretion in rainbow trout (Oncorhynchus mykiss) during early life stages
... for urea synthesis in teleost fish is uricolysis and/or arginolysis, as most species lack a functional OUC (Goldstein and Forster, 1965; Huggins et ...to urea (see ...to urea via ...
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Developmental Outcomes With Early Orthotopic Liver Transplantation for Infants With Neonatal-Onset Urea Cycle Defects and a Female Patient With Late-Onset Ornithine Transcarbamylase Deficiency
... UCD, urea cycle defect; CPS, carbamoyl phos- phate synthase; OTC, ornithine transcarbamylase; EBV, Epstein- Barr virus; OLT, orthotopic liver ...the urea cycle are character- ized by ...
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The Urea Cycle. April 11, 2003 Bryant Miles
... With a deficiency of CPSI or ornithine transcarbamylase the ability to synthesize citrulline is impaired. Under these conditions excess ammonia accumulates in the liver and kidneys as glycine and glutamine. The ...
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Suggested guidelines for the diagnosis and management of urea cycle disorders
... Abstract Urea cycle disorders (UCDs) are inborn errors of ammonia detoxification/arginine synthesis due to defects affecting the catalysts of the Krebs-Henseleit cycle (five core enzymes, one ...
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Suggested guidelines for the diagnosis and management of urea cycle disorders
... Abstract Urea cycle disorders (UCDs) are inborn errors of ammonia detoxification/arginine synthesis due to defects affecting the catalysts of the Krebs-Henseleit cycle (five core enzymes, one ...
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Urea Cycle Defects: A Challenge for Neonatologists in Limited Resource Settings
... the urea cycle enzymes including deficiency of carbamoyl phosphate synthetase 1 (CPS1), ornithine transcarbamylase (OTC), argininosuccinate synthetase (ASS), argininosuccinate lyase (ASL) and ...
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Vaccines Are Not Associated With Metabolic Events in Children With Urea Cycle Disorders
... ornithine transcarbamylase defi- ciency (most common); carbamoyl phosphate synthetase I deficiency; argininosuccinic acid synthetase deficiency or citrullinemia; arginino- succinic acid lyase deficiency or ...
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Isolated Hepatocyte Transplantation in an Infant With a Severe Urea Cycle Disorder
... ABBREVIATIONS. OTC, ornithine transcarbamylase; DV, ductus venosus; OLT, orthotopic liver transplantation; IV, intravenous. I solated hepatocyte transplantation has been shown to be effective in restoring ...
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A Role for Cytosolic Fumarate Hydratase in Urea Cycle Metabolism and Renal Neoplasia
... the urea cycle, we hypothesized that transient arginine depletion might selectively inhibit the growth of FH-deficient ...and urea by arginase (Shambaugh, 1977), the effectiveness and time course of ...
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A Role for Cytosolic Fumarate Hydratase in Urea Cycle Metabolism and Renal Neoplasia
... the urea cycle, we hypothesized that transient arginine depletion might selectively inhibit the growth of FH-deficient ...and urea by arginase (Shambaugh, 1977), the effectiveness and time course of ...
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Urea cycle disorders in Argentine patients: clinical presentation, biochemical and genetic findings
... was downstream of CPS1, thus ruling out possible CPS1D or NAGSD. Ornithine transcarbamylase deficiency A total of 26 patients belonging to 12 families were diag- nosed with OTCD; 17/26 were women and 9/26 were ...
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Urea cycle disorders in India: clinical course, biochemical and genetic investigations, and prenatal testing
... Background: Urea cycle disorders (UCDs) are inherited metabolic disorders that present with hyperammonemia, and cause significant mortality and morbidity in infants and ...patients. Ornithine ...
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Efficacy and safety of i.v. sodium benzoate in urea cycle disorders: a multicentre retrospective study
... 22 ornithine transcarbamylase (20 confirmed, 2 suspected), 18 arginino-succinate synthetase, 15 carbamoyl phosphate synthetase, 3 arginosuccinate lyase, 1 arginase deficiency, 1 N-acetylglutamate synthetase, 1 HHH ...
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Hyperammonemia due to urea cycle disorders: a potentially fatal condition in the intensive care setting
... deficiency. Ornithine translocase deficiency is a rare disease that results in hyperornithi- nemia, homocitrullinuria, and hyperammonemia, similar to those in ...in ornithine transport into the mitochondria ...
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Urea cycle disorders in Spain: an observational, cross-sectional and multicentric study of 104 cases
... The urea cycle is the final common pathway for the excre- tion of waste nitrogen as well as arginine synthesis ...[1,2]. Urea cycle disorders (UCDs) are caused by a deficiency of one of the ...
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Cross-sectional observational study of 208 patients with non-classical urea cycle disorders.
... on all patients with non-classical UCDs treated by the par- ticipants in 20 European metabolic centres. Information was collected on a cohort of 208 patients 50% of which were ≥ 16 years old. The largest subgroup (121 ...
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Case Report A novel mutation in ornithine transcarbamylase gene identified from a Chinese child with ornithine transcarbamylase deficiency
... mutation, ornithine transcarbamylase deficiency Introduction Ornithine transcarbamylase deficiency (OTCD) is the most common type of congenital urea cycle disorders [1, 2] and is an X-linked ...
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Metabolic reprogramming of the urea cycle pathway in experimental pulmonary arterial hypertension rats induced by monocrotaline
... to ornithine and urea by ...aminoacylase-1. Ornithine is then converted to polyamines and proline, which are involved in the proliferation of pulmonary arterial smooth muscle cells and collagen ...
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Clinical course of 63 patients with neonatal onset urea cycle disorders in the years 2001–2013
... Keywords: Urea cycle disorders, neonatal hyperammonemia, hyperammonemic crisis, Carbamoyl phosphate synthetase 1 deficiency, Ornithine transcarbamylase deficiency, Argininosuccinate synthetase ...
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