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Titolo:
Phenylketonuria: Tyrosine beyond the phenylalanine-restricted diet
Autore:
van Spronsen, FJ; Smit, PGA; Koch, R;
Indirizzi:
Univ Groningen Hosp, Beatrix Children Hosp, Dept Metab Dis, NL-3700 RB Groningen, Netherlands Univ Groningen Hosp Groningen Netherlands NL-3700 RB ningen, Netherlands Childrens Hosp, Div Med Genet, Los Angeles, CA 90027 USA Childrens Hosp Los Angeles CA USA 90027 Genet, Los Angeles, CA 90027 USA
Titolo Testata:
JOURNAL OF INHERITED METABOLIC DISEASE
fascicolo: 1, volume: 24, anno: 2001,
pagine: 1 - 4
SICI:
0141-8955(200102)24:1<1:PTBTPD>2.0.ZU;2-1
Fonte:
ISI
Lingua:
ENG
Soggetto:
MATERNAL PHENYLKETONURIA; SUPPLEMENTATION; ISOLEUCINE; LEUCINE; VALINE; BRAIN; VIGILANCE; DOPAMINE; CHILDREN; DEFICITS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
25
Recensione:
Indirizzi per estratti:
Indirizzo: van Spronsen, FJ Univ Groningen Hosp, Beatrix Children Hosp, Dept Metab Dis, POB 30001, NL-3700 RB Groningen, Netherlands Univ Groningen Hosp POB 30001 Groningen Netherlands NL-3700 RB
Citazione:
F.J. van Spronsen et al., "Phenylketonuria: Tyrosine beyond the phenylalanine-restricted diet", J INH MET D, 24(1), 2001, pp. 1-4

Abstract

Controversies exist on the role of tyrosine in the pathogenesis of phenylketonuria (PKU) and, consequently, on the therapeutic role of tyrosine. Thisreview examines data and theoretical considerations on the role of tyrosine in the pathogenesis and treatment of PKU. It is concluded that treatment with tyrosine alone to replace the phenylalanine-restricted diet cannot be justified. A treatment with large neutral amino acids (LNAA) including tyrosine to restore the balance in the transport of phenylalanine and other LNAA across the blood-brain barrier deserves further investigation. Such studies should prove the safety and the efficacy of such a treatment, finding the optimal dose of all LNAA, disclosing the correct age to start and the wayto monitor treatment biochemically.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 01/04/20 alle ore 11:49:12