Results 201 to 210 of about 7,599 (237)

Effect of insulin on hyperphenylalaninemia rats

Biochemical Medicine, 1979
Abstract The influence of insulin on hydroxylation and decarboxylation of tyrosine was examined in control and hyper-Phe rats. Insulin elevated brain tyrosine hydroxylase activity in both control and hyper-Phe rats. Insulin administration also increased the decarboxylation of tyrosine in the brain and liver of control rats.
Toshikiyo Shohmori, Mutsutoshi Kohsaka, Misako Okita   +2 more
openaire   +3 more sources

Maternal non-phenylketonuric mild hyperphenylalaninemia

European Journal of Pediatrics, 1996
Unlike maternal phenylketonuria (PKU) which produces severe birth defects when untreated during pregnancy, maternal non-PKU mild hyperphenylalaninemia (MHP) has a less severe impact but whether it is benign or may have long-term consequences for offspring has been unclear.
F. de la Cruz, Susan E. Waisbren, Alan Leviton, Elizabeth N. Allred, D. Lobbregt, Harvey L. Levy, Richard Koch, Bobbye Rouse, Reuben Matalon, William B. Hanley   +9 more
openaire   +3 more sources

Dihydroxanthopterinuria in phenylketonuria and lethal hyperphenylalaninemia patients

Clinica Chimica Acta, 1977
7,8-Dihydroxanthopterin in identified in urine from phenylketonuria and lethal hyperphenylalaninemia patients. Because 7,8-dihyroxanthopterin is readily oxidised to xanthopterin, most of the characterisation was performed on xanthopterin. This finding indicates a gross disturbance of tetrahydrobiopterin metabolism in these patients.
B.M. Watson, Richard G. H. Cotton, P. Schlensinger   +2 more
openaire   +3 more sources

Antioxidant treatment strategies for hyperphenylalaninemia

Metabolic Brain Disease, 2013
Hyperphenylalaninemia (HPA) leads to increased oxidative stress in patients with phenylketonuria (PKU) and in animal models of PKU. Early diagnosis and immediate adherence to a phenylalanine-restricted diet prevents HPA and, consequently, severe brain damage.
George A. Karikas, Carlos Severo Dutra-Filho, Priscila Nicolao Mazzola, Kleopatra H. Schulpis   +3 more
openaire   +3 more sources

Nutrition in pregnancy of women with hyperphenylalaninemia

Journal of the American Dietetic Association, 1982
This study suggests that, with appropriate diet manipulation and control of blood phenylalanine, women with PKU can give birth to normal infants.
Harriet H. Cloud, Hazel Stroud, M. Blaskovics, Elaine W. Lis, Phyllis B. Acosta, Phyllis B. Acosta, Elizabeth Wenz   +6 more
openaire   +3 more sources

Spotlight on Sapropterin in Primary Hyperphenylalaninemia†

BioDrugs, 2009
Sapropterin dihydrochloride (Kuvan)) is a synthetic formulation of the active 6R-isomer of tetrahydrobiopterin, a naturally occurring co-factor for phenylalanine hydroxylase. In the EU, sapropterin is approved for the treatment of hyperphenylalaninemia in patients > or =4 years of age with tetrahydrobiopterin-responsive phenylketonuria (PKU), and in ...
Gillian M. Keating, Mark Sanford
openaire   +3 more sources

Studies on experimentally induced hyperphenylalaninemia

Journal of Intellectual Disability Research, 1988
ABSTRACT.The administration of several compounds thai can increase plasma phcnylalanine levels and or inhibit phcnylalanine hydroxylase in rats was studied in order to determine iheir usefulness in inducing a phenylketirtiuria‐like state. The results of this investigation revealed that 4′5 um/10 g p‐chlorophenylalanine is more el'lective lhan L ...
R. Ellenbogen, Siegfried M. Pueschel, Joan M. Boylan   +2 more
openaire   +3 more sources

Hyperphenylalaninemia due to impaired dihydrobiopterin biosynthesis

European Journal of Pediatrics, 1981
A fourteen month-old boy with atypical phenylketonuria was treated with 5-hydroxytryptophan, L-dopa and peripheral aromatic amino acid decarboxylase inhibitor (Ro 4-4602:benserazide). Despite the good control of plasma phenylalanine on a low phenylalanine diet, he had shown no improvement in his development but progressive neurological symptoms, such ...
Tomofusa Usui, Kazuo Iwai, Masahiro Kohashi, T. Tanaka, H. Yoshida, K. Tomita, N. Arai, K. Aihara, Kuniaki Narisawa   +8 more
openaire   +3 more sources

SEROTONIN DEFICIENCY IN EXPERIMENTAL HYPERPHENYLALANINEMIA

Journal of Neurochemistry, 1974
Abstract— The mechanism of serotonin depletion was studied in the preweanling rat in which a chemical simulation of phenylketonuria had been induced by injections of p‐CPA and l‐PA. Experimental conditions were selected to effectively minimize the contribution by deficient tryptophan hydroxylation and 5‐HTP transport.
openaire   +3 more sources

THE HYPERPHENYLALANINEMIAS OF MAN AND MOUSE

Annual Review of Genetics, 1994
Charles R. Scriver, Seymour Kaufman, Randy C. Eisensmith, Savio L. C. Woo   +3 more
openaire   +3 more sources