�ber den Aminos�uren-Transport bei phenylketonurischer Oligophrenie

Linneweh, F.; Ehrlich, M.; Graul, E.H.; Hundeshagen, H.

doi:10.1007/bf01483387

Cited by 35 publications

(7 citation statements)

References 4 publications

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“…This theory is supported by the observation that orally administered radioactively labelled amino acids (arginine and leucine) showed strongly impaired uptake into plasma in untreated PKU patients [24]. The normal intestinal absorbtion was restored when the plasma level of phenylalanine was normalized by phenylalanine-restricted diet [24]. The high level of phenylalanine in the bile and the digestive fluids might be responsible for a competitive inhibition of the intestinal absorbtion of tyrosine, tryptophan and other amino acids [37,50] resulting in loss of tyrosine and tryptophan in the stools of untreated PKU patients [50].…”

Section: Discussionmentioning

confidence: 74%

“…A reduced intestinal absorption may also contribute to the decrease in plasma levels of tyrosine and tryptophan. This theory is supported by the observation that orally administered radioactively labelled amino acids (arginine and leucine) showed strongly impaired uptake into plasma in untreated PKU patients [24]. The normal intestinal absorbtion was restored when the plasma level of phenylalanine was normalized by phenylalanine-restricted diet [24].…”

Section: Discussionmentioning

confidence: 90%

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Increased neurotransmitter biosynthesis in phenylketonuria induced by phenylalanine restriction or by supplementation of unrestricted diet with large amounts of tyrosine

Lykkelund¹,

Nielsen

et al. 1988

Eur J Pediatr

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Seven phenylketonuria (PKU) patients aged 15-24 years were allowed unrestricted diet for 3 weeks. Three of these patients performed well on unrestricted diet according to visual reaction time variability (RTv 50-100 ms) and did not show significant changes when returning to the phenylalanine-restricted diet (RTv 70-100 ms). Neither did the concentrations of homovanillic acid (HVA) and 5-hydroxyindoleacetic acid (5-HIAA) in cerebrospinal fluid (CSF) change significantly. Four of the patients, however, performed rather poorly (RTv 120-220 ms) on unrestricted diet and improved significantly (P less than 0.03) when the diet was restored (RTv 70-150 ms). The improvements were accompanied by significant (P less than 0.01 and P less than 0.02) increases (mean 52% and 109%) in CSF levels of HVA and 5-HIAA. Five PKU patients aged 15-23 years were allowed unrestricted diet or unrestricted diet supplemented with various amounts of tyrosine (106-194 mg/kg per 24 h). Two of these patients performed very well on unrestricted diet (RTv 60 ms) and showed little change when the unrestricted diet was supplemented with tyrosine (RTv 70 ms and 80 ms). The three other patients, who performed rather poorly (RTv 120-220 ms), improved significantly (P less than 0.03) when the unrestricted diet was supplemented with tyrosine (RTv 70-140 ms). HVA in CSF increased significantly (P less than 0.01) with the tyrosine supplement when the amount exceeded a threshold of approximately 80 mg/kg per 24 h.(ABSTRACT TRUNCATED AT 250 WORDS)

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Section: Discussionmentioning

confidence: 74%

Section: Discussionmentioning

confidence: 90%

Increased neurotransmitter biosynthesis in phenylketonuria induced by phenylalanine restriction or by supplementation of unrestricted diet with large amounts of tyrosine

Lykkelund¹,

Nielsen

et al. 1988

Eur J Pediatr

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“…A load of a single amino acid consistently results in an elevation of the plasma level of the administered amino acid while simulta neously depressing plasma levels of other amino acids [5,6], Conflicting results, how ever, have been reported on the effect of ingestion of a mixture of amino acids upon blood amino acid concentrations.…”

Section: Discussionmentioning

confidence: 76%

Plasma Amino Acid Pattern at Noon in Early Treated Hyperphenylalaninemic, Phenylketonuric, and Normal Children

Held¹,

Koepp²,

Plettner³

et al. 1983

Ann Nutr Metab

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In 41 hyperphenylalaninemic early treated children (classical phenylketonurics and hyperphenylalaninemic variants) on diet, aged 2 months to 15 years, the plasma amino acid pattern at noon was compared with that in 31 normal age-matched controls. In contrast to previously published results in infants, the mean levels of single amino acids deviated, with the exception of phenylalanine, only slightly from that of normal controls in patients of all five age groups investigated. Constant findings were a tendency towards low mean levels of arginine in younger patients and elevated levels of lysine, serine, and histidine which were found in patients of all age groups.

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“…In diesem Zusammenhang wichtig ist die Feststellung von Linneweh u. Mitarb. [12], daß bei Phenylke tonurie nach oraler Applikation von markiertem Arginin oder Leucin die A ktivitäten im Plasma wesentlich niedriger sind als bei Ver gleichspersonen, was die Autoren als Beweis für eine verzögerte Re sorption ansehen; diätetische Einstellung und Normalisierung des Phenylalaninspiegels normalisierte die Verhältnisse.…”

Section: Die Resorption Der Aminosäuren Und Ihre Störungenunclassified

Enterale Eiweißresorption

Zöllner¹

1965

Digestion

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�ber den Aminos�uren-Transport bei phenylketonurischer Oligophrenie

Cited by 35 publications

References 4 publications

Increased neurotransmitter biosynthesis in phenylketonuria induced by phenylalanine restriction or by supplementation of unrestricted diet with large amounts of tyrosine

Increased neurotransmitter biosynthesis in phenylketonuria induced by phenylalanine restriction or by supplementation of unrestricted diet with large amounts of tyrosine

Plasma Amino Acid Pattern at Noon in Early Treated Hyperphenylalaninemic, Phenylketonuric, and Normal Children

Enterale Eiweißresorption

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