A new experimental model of hyperphenylalaninemia in rat. Effect of p-chlorophenylalanine and cotrimoxazole

Dhondt, Jean-Louis; Dautrevaux, M; Biserte, G; Farriaux, J. P.

doi:10.1016/s0300-9084(77)80250-2

Cited by 9 publications

(2 citation statements)

References 17 publications

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“…p -chlorophenylalanine along with phenylalanine itself [89, 90]. This could mimic the PKU phenotype and appeared crucial to decipher the PKU pathomechanism, but was inappropriate for the development of gene therapy based therapeutics because of the missing genetic defect along with potential side effects of the PAH inhibitor [89, 91].…”

Section: Amino Acid Metabolism Disordermentioning

confidence: 99%

Gene Therapy for the Treatment of Neurological Disorders: Metabolic Disorders

Gessler

Gao

2016

Methods in Molecular Biology

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Metabolic disorders comprise a large group of heterogeneous diseases ranging from very prevalent diseases such as diabetes mellitus to rare genetic disorders like Canavan Disease. Whether either of these diseases is amendable by gene therapy depends to a large degree on the knowledge of their pathomechanism, availability of the therapeutic gene, vector selection, and availability of suitable animal models. In this book chapter, we review three metabolic disorders of the central nervous system (CNS; Canavan Disease, Niemann–Pick disease and Phenylketonuria) to give examples for primary and secondary metabolic disorders of the brain and the attempts that have been made to use adeno-associated virus (AAV) based gene therapy for treatment. Finally, we highlight commonalities and obstacles in the development of gene therapy for metabolic disorders of the CNS exemplified by those three diseases.

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Section: Amino Acid Metabolism Disordermentioning

confidence: 99%

Gene Therapy for the Treatment of Neurological Disorders: Metabolic Disorders

Gessler

Gao

2016

Methods in Molecular Biology

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“…These investigators found u-methylphenylalanine to be a better compound in producing a phenylketonuria-like state in rats. Experiments with cotrimoxazole which inhibits dihydropteridine reductase were carried out by Dhondt et al (1977). They observed that the administration of cotrimoxazole together with p-chlorophenylalanine increases serum phenylalanine levels and decreases phenylalanine hydroxylase activity.…”

Section: Introductionmentioning

confidence: 99%

Studies on experimentally induced hyperphenylalaninemia

Pueschel

Boylan

Ellenbogen

1988

J intellect Disabil Res

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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‐phcnylalaninc, a‐methylphenylalanine, trimelhoprim, Hactrim and Scptra, since the lormer auii Mukl julidiiced both adequate hyperphenylalaninemia and marked inhibition of hepatic phenylalanine hydroxylase activity. In addition, a 24‐h study provided important insights into the changing diurnal patterns of specified biochemical parameters.

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Phenylketonuria

Lane

Neuhoff

1980

Naturwissenschaften

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Phenylketonuria is a genetic defect that leads to imbecility, if the diagnosis is not made directly after birth. Since the development of imbecility can be almost totally halted by suitable dietary treatment, phenylketonuria is of more interest to neurochemists than to clinicians. This genetic defect is not known to occur in aminals. It is therefore necessary to develop suitable models for neurochemical analysis. Most successful is the simultaneous application to developing rats of alpha-methyl-phenylalanine (an inhibitor of phenylalanine hydroxylase), together with phenylalanine. With this treatment it is possible to induce changes in the central nervous system which are surprisingly similar to those found in patients with phenylketonuria. This model is therefore of great importance in the analyses of the disturbances of metabolism, which finally causes the severe defects in normal brain function.

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A new experimental model of hyperphenylalaninemia in rat. Effect of p-chlorophenylalanine and cotrimoxazole

Cited by 9 publications

References 17 publications

Gene Therapy for the Treatment of Neurological Disorders: Metabolic Disorders

Gene Therapy for the Treatment of Neurological Disorders: Metabolic Disorders

Studies on experimentally induced hyperphenylalaninemia

Phenylketonuria

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