caa a22 4500 469045329 CHVBK 20180323132822.0 cr unu---uuuuu 170328e19920301xx s 000 0 eng 10.1007/BF01799638 doi (NATIONALLICENCE)springer-10.1007/BF01799638 Myelin turnover in hyperphenylalaninaemia. A re-evaluation with the HPH-5 mouse [Elektronische Daten] [F. Hommes, L. Moss] Summary: Myelin turnover has been studied in the 25-day-old HPH-5 mouse, a phenylalanine hydroxylase-deficient mouse mutant. The half-life of the fast component of myelin decreased from 15 days in control mice to 4.5 days at blood phenylalanine levels of 2.5 mmol/L. The slow component of myelin seems also to be affected by the high phenylalanine level. These observations confirm similar observations obtained with chemically induced models of hyperphenylalaninaemia and are therefore due to the hyperphenylalaninaemiaper se, independently of the inhibitors of phenylalanine hydroxylase. An intermediate blood level of phenylalanine (0.7 mmol/L) likewise seems to interfere with myelin metabolism, although to a lesser degree. SSIEM and Kluwer Academic Publishers, 1992 Hommes F. Department of Biochemistry and Molecular Biology, Medical College of Georgia, 30912-2100, Augusta, GA, USA aut Moss L. Department of Biochemistry and Molecular Biology, Medical College of Georgia, 30912-2100, Augusta, GA, USA aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/2(1992-03-01), 243-251 0141-8955 15:2<243 1992 15 10545 https://doi.org/10.1007/BF01799638 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF01799638 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Hommes F. Department of Biochemistry and Molecular Biology, Medical College of Georgia, 30912-2100, Augusta, GA, USA aut NATIONALLICENCE 700 1- Moss L. Department of Biochemistry and Molecular Biology, Medical College of Georgia, 30912-2100, Augusta, GA, USA aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/2(1992-03-01), 243-251 0141-8955 15:2<243 1992 15 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer