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Literature DB >> 6796768

Organic acidaemia and Hyperammonaemia: review.

Abstract

Recent clinical and experimental evidence on the effects of organic acids in producing or ameliorating hyperammonaemia is reviewed. The importance of hepatic mitochondrial N-acetylglutamate and its precursors, glutamate and acetyl-CoA, in the control of ureagenesis and thus blood ammonia levels is emphasized by recent work. The hypothesis is proposed that protein loads stimulated urea cycle activity via glutamate-induced changes in N-acetylglutamate concentration, while the effects of organic acids on ureagenesis are related in a predictable way to their effects on hepatic concentrations of acetyl-CoA.

Entities: Chemical Disease

Mesh：

Substances：

Year: 1981 PMID： 6796768 DOI： 10.1007/BF02263648

Source DB: PubMed Journal: J Inherit Metab Dis ISSN： 0141-8955 Impact factor: 4.982

16 in total

1. Treatment of carbamyl phosphate synthetase deficiency with keto analogues of essential amino acids.

Authors: M Batshaw; S Brusilow; M Walser
Journal: N Engl J Med Date: 1975-05-22 Impact factor: 91.245

2. Turnover of carbamyl-phosphate synthase, of other mitochondrial enzymes and of rat tissues. Effect of diet and of thyroidectomy.

Authors: M Nicoletti; C Guerri; S Grisolia
Journal: Eur J Biochem Date: 1977-05-16

3. Jamaican vomiting sickness and Reye's syndrome.

Authors: D A Trauner; W L Nyhan; L Sweetman
Journal: N Engl J Med Date: 1976-12-23 Impact factor: 91.245

4. Role of acetylglutamate in ureotelism. Variations in acetylglutamate level and its possible significance in control of urea synthesis in mammalian liver.

Authors: K Shigesada; K Aoyagi; M Tatibana
Journal: Eur J Biochem Date: 1978-04-17

5. Use of keto acids in inborn errors of urea synthesis.

Authors: S Brusilow; M Batshaw; M Walser
Journal: Curr Concepts Nutr Date: 1979

6. Rate-limiting steps in metabolic pathways.

Authors: R Rognstad
Journal: J Biol Chem Date: 1979-03-25 Impact factor: 5.157

7. Effects of branched chain alpha-ketoacids on the metabolism of isolated rat liver cells. II. Interactions with gluconeogenesis and urea synthesis.

Authors: E Wałajtys-Rode; K E Coll; J R Williamson
Journal: J Biol Chem Date: 1979-11-25 Impact factor: 5.157

2. Severe Hyperammonemic Encephalopathy Requiring Dialysis Aggravated by Prolonged Fasting and Intermittent High Fat Load in a Ramadan Fasting Month in a Patient with CPTII Homozygous Mutation.

Authors: P Phowthongkum; C Ittiwut; V Shotelersuk
Journal: JIMD Rep Date: 2017-11-21

2 in total

Organic acidaemia and Hyperammonaemia: review.

1. Treatment of carbamyl phosphate synthetase deficiency with keto analogues of essential amino acids.

2. Turnover of carbamyl-phosphate synthase, of other mitochondrial enzymes and of rat tissues. Effect of diet and of thyroidectomy.

3. Jamaican vomiting sickness and Reye's syndrome.

4. Role of acetylglutamate in ureotelism. Variations in acetylglutamate level and its possible significance in control of urea synthesis in mammalian liver.

5. Use of keto acids in inborn errors of urea synthesis.

6. Rate-limiting steps in metabolic pathways.

7. Effects of branched chain alpha-ketoacids on the metabolism of isolated rat liver cells. II. Interactions with gluconeogenesis and urea synthesis.

8. The use of ornithine salts of branched-chain ketoacids in portal-systemic encephalopathy.

9. Dysautonomia in an infant with secondary hyperammonemia due to propionyl coenzyme A carboxylase deficiency.

10. Studies of the cause and treatment of hyperammonemia in females with ornithine transcarbamylase deficiency.

1. Hudson memorial lecture. Neonatal management of organic acidurias. Clinical update.

2. Severe Hyperammonemic Encephalopathy Requiring Dialysis Aggravated by Prolonged Fasting and Intermittent High Fat Load in a Ramadan Fasting Month in a Patient with CPTII Homozygous Mutation.