Alternative pathway therapy for urea cycle disorders

Journal of Inherited Metabolic Disease

Volumn 21, Issue SUPPL. 1, 1998, Pages 101-111

  Feillet, F ^a   Leonard, J V ^a  

^a UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

NITROGEN;

CONFERENCE PAPER; HOMEOSTASIS; HUMAN; INBORN ERROR OF METABOLISM; NITROGEN METABOLISM; PROTEIN RESTRICTION; UREA CYCLE;

EID: 0031879761     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1023/a:1005365825875     Document Type: Article

References (9)

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2. Bachmann C, Lüthi H, Gradwohl M, Colombo JP (1986) Brain uptake of tryptophan in urease-injected hyperammonaemic rats after treatment with benzoate or hippurate. Biochem Med Metab Biol 36: 214-219.
3. Barshop BA, Breuer J, Holm J, Leslie J, Nyhan WL (1989) Excretion of hippuric acid during sodium benzoate therapy in patients with hyperglycinaemia or hyperammonaemia. J Inher Metab Dis 12: 72-79.
4. Batshaw ML (1983) Sodium benzoate and arginine: alternative pathway therapy in inborn errors of urea synthesis. Prog Clin Biol Res 127: 69-83.
5. Batshaw ML, Brusilow SW (1981) Evidence of lack of toxicity of sodium phenylacetate and sodium benzoate in treating urea cycle enzymopathies. J Inher Metab Dis 4: 231.
6. Brusilow SW (1984) Arginine, an indispensable amino acid for patients with inborn errors of urea synthesis. J Clin Invest 74: 2144-2148.
7. Brusilow SW (1991) Phenylacetylglutamine may replace urea as a vehicle for waste nitrogen excretion. Pediatr Res 29: 147-150.
8. Brusilow SW, Batshaw ML (1979) Arginine treatment of argininosuccinase deficiency. Lancet 1: 124-126.
9. Brusilow SW, Horwich AL (1995) Urea cycle enzymes. In. Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The Metabolic and Molecular Bases of Inherited Disease, 7th edn. New York: McGraw-Hill, 1187-1232.