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Neuropediatrics 1982; 13(2): 88-92
DOI: 10.1055/s-2008-1059603
© Georg Thieme Verlag KG Stuttgart · New York

Impaired Permeability of the Blood-Cerebrospinal Fluid Barrier in Hyperphenylalaninaemia1

N.  Piel , J. D. Lane , G.  Hüther , V.  Neuhoff
  • Max-Planck-Institut für experimentelle Medizin, Forschungsstelle Neurochemie, Hermann-Rein-Str. 3,3400 Göttingen, FRG
1 This research was supported by the Deutsche Forschungsgemeinschaft, SFB 33.
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Publication History

Publication Date:
19 March 2008 (online)

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Abstract

In hyperphenylalaninaemic rats the accumulation of 5-hydroxytryptophan (5-HTP) in the cerebrospinal fluid (CSF) could be shown in spite of the fact, that the 5-HTP levels of serum and brain remain constant. In vitro studies of the influx and efflux of 5-HTP and phenylalanine on isolated beef choroid plexus suggested that both amino acids use the same carrier system. It is concluded that a high concentration of phenylalanine inhibits the re-uptake of 5-HTP by the endothelial cells of the choroid plexus. Additionally, an increased efflux of 5-HTP from choroid plexus leads to the accumulation of 5-HTP in the cerebrospinal fluid.

Key words

Phenylketonuria - Hyperphenylalaninaemia - Cerebrospinal fluid - Liquor - Indoles - 5-hydroxytryptophan