Nutritional therapy improves growth and protein status of children with a urea cycle enzyme defect

2005 ◽  
Vol 86 (4) ◽  
pp. 448-455 ◽  
Author(s):  
Phyllis B. Acosta ◽  
Steven Yannicelli ◽  
Alan S. Ryan ◽  
Georgianne Arnold ◽  
Barbara J. Marriage ◽  
...  
Keyword(s):  
Urea Cycle ◽  
Nutritional Therapy ◽  
Enzyme Defect ◽  
Cycle Enzyme ◽  
Protein Status ◽  
Urea Cycle Enzyme

Corrigendum to “Nutritional therapy improves growth and protein status of children with a urea cycle enzyme defect” [Mol. Genet. Metab. 86 (2005) 448–455]

2006 ◽  
Vol 89 (3) ◽  
pp. 288
Author(s):  
Phyllis B. Acosta ◽  
Steven Yannicelli ◽  
Alan S. Ryan ◽  
Georgianne Arnold ◽  
Barbara J. Marriage ◽  
...  
Keyword(s):  
Urea Cycle ◽  
Nutritional Therapy ◽  
Enzyme Defect ◽  
Cycle Enzyme ◽  
Protein Status ◽  
Urea Cycle Enzyme

scholarly journals Transient hyperammonaemia in an adult German shepherd dog : case report

1997 ◽  
Vol 68 (2) ◽  
Author(s):  
R.G. Lobetti ◽  
D.B. Miller ◽  
T. Dippenaar
Keyword(s):  
Case Report ◽  
Liver Disease ◽  
Urinary Tract ◽  
Drug Therapy ◽  
Urea Cycle ◽  
Urinary Tract Obstruction ◽  
German Shepherd ◽  
Cycle Enzyme ◽  
German Shepherd Dog ◽  
Urea Cycle Enzyme

A 3-year-old male German shepherd dog was presented with severe generalised seizures. The dog was protein-intolerant and showed severe hyperammonaemia on ammonia stimulation. The hyperammonaemic state was present for at least 6 weeks and then spontaneously resolved. No obvious cause (liver disease, portocaval shunts, urea cycle enzyme deficiencies, drug therapy or urinary tract obstruction) could be identified. It is possible that this dog had a variation of transient hyperammonaemic syndrome, described in man and recently in a juvenile Irish wolfhound, that extended into adulthood.

scholarly journals Long-chain fatty acids suppress the induction of urea cycle enzyme genes by glucocorticoid action

FEBS Letters ◽  
1996 ◽  
Vol 399 (3) ◽  
pp. 310-312 ◽  
Author(s):  
Mineko Tomomura ◽  
Akito Tomomura ◽  
Dewan Md. Abdullah Abu Musa ◽  
Takeyori Saheki
Keyword(s):  
Fatty Acids ◽  
Urea Cycle ◽  
Long Chain Fatty Acids ◽  
Cycle Enzyme ◽  
Urea Cycle Enzyme ◽  
Glucocorticoid Action ◽  
Long Chain ◽  
Chain Fatty Acids

scholarly journals Clinical Consequences of Urea Cycle Enzyme Deficiencies and Potential Links to Arginine and Nitric Oxide Metabolism

2004 ◽  
Vol 134 (10) ◽  
pp. 2775S-2782S ◽  
Author(s):  
Fernando Scaglia ◽  
Nicola Brunetti-Pierri ◽  
Soledad Kleppe ◽  
Juan Marini ◽  
Susan Carter ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Urea Cycle ◽  
Cycle Enzyme ◽  
Nitric Oxide Metabolism ◽  
Clinical Consequences ◽  
Urea Cycle Enzyme

Urea Cycle Enzyme Adaptation to Dietary Protein in Primates

Science ◽  
1971 ◽  
Vol 172 (3987) ◽  
pp. 1042-1043 ◽  
Author(s):  
C. T. Nuzum ◽  
P. J. Snodgrass
Keyword(s):  
Dietary Protein ◽  
Urea Cycle ◽  
Cycle Enzyme ◽  
Enzyme Adaptation ◽  
Urea Cycle Enzyme

scholarly journals Mice deficient in the urea-cycle enzyme, carbamoyl phosphate synthetase i, die during the early neonatal period from hyperammonemia

Hepatology ◽  
1999 ◽  
Vol 29 (1) ◽  
pp. 181-185 ◽  
Author(s):  
J. Paul Schofield ◽  
J. Paul Schofield ◽  
Timothy M. Cox ◽  
C. Thomas Caskey ◽  
Maki Wakamiya
Keyword(s):  
Urea Cycle ◽  
Neonatal Period ◽  
Carbamoyl Phosphate Synthetase ◽  
Carbamoyl Phosphate ◽  
Cycle Enzyme ◽  
Early Neonatal Period ◽  
Urea Cycle Enzyme
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