Literature DB >> 1491633

Serotonin-GABA treatment is hypothesized for self-injury in Lesch-Nyhan syndrome.

A Gedye.   

Abstract

The self-injurious movements of Lesch-Nyhan patients are considered extremely severe and currently intractable. Lesch-Nyhan syndrome is a genetic disorder of purine metabolism resulting in several abnormalities, one of which is elevated levels of xanthine. The author reasons that elevated plasma xanthine sets off a chain of events that produce excessive endogenous convulsants and diminished endogenous anticonvulsants. Treatment is proposed for self-injury in Lesch-Nyhan that entails reducing production of two endogenous convulsants (kynurenine and quinolinic acid) and enhancing two endogenous anticonvulsants (serotonin and gamma aminobutyric acid).

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Year:  1992        PMID: 1491633     DOI: 10.1016/0306-9877(92)90026-9

Source DB:  PubMed          Journal:  Med Hypotheses        ISSN: 0306-9877            Impact factor:   1.538


  2 in total

1.  Intrastriatal injection of hypoxanthine reduces striatal serotonin content and impairs spatial memory performance in rats.

Authors:  Caren Serra Bavaresco; Fabria Chiarani; Eduardo Duringon; Marcelo Machado Ferro; Cláudio Da Cunha; Carlos Alexandre Netto; Angela Terezinha de Souza Wyse
Journal:  Metab Brain Dis       Date:  2007-01-13       Impact factor: 3.584

2.  HPRT-deficiency dysregulates cAMP-PKA signaling and phosphodiesterase 10A expression: mechanistic insight and potential target for Lesch-Nyhan Disease?

Authors:  Ghiabe-Henri Guibinga; Fiona Murray; Nikki Barron
Journal:  PLoS One       Date:  2013-05-14       Impact factor: 3.240

  2 in total

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