title: Genetic and pharmacological suppression of polyglutamine-dependent neuronal dysfunction in Caenorhabditis elegans.
abstract: The identification of disease genes for several neurodegenerative illnesses has allowed for the development of disease models in experimental organisms. We discuss our approach to studying Huntington's disease, the best characterized of the polyglutamine (polyQ) expansion disorders. We have developed a system in Caenorhabditis elegans to study the effects of (polyQ)-dependent neuronal dysfunction at the resolution of two neurons in screening for genetic and pharmacological suppression. Our data suggest that C. elegans might be instructive in searching for targets and active compounds against polyglutamine neuronal toxicity.
authors.affiliation: Laboratory of Genomic Biology, Inserm Avenir Group, Centre Paul Broca, 75014 Paris, France.
authors.forename: J Alex
authors.initials: JA
authors.lastname: Parker
authors.forename: Sébastien
authors.initials: S
authors.lastname: Holbert
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Lambert
authors.forename: Salima
authors.initials: S
authors.lastname: Abderrahmane
authors.forename: Christian
authors.initials: C
authors.lastname: Néri
mesh_terms: D000818:Animals; D017173:Caenorhabditis elegans / Q000235:genetics / Q000378:metabolism*; D004195:Disease Models, Animal; D004353:Drug Evaluation, Preclinical / Q000379:methods / Q000639:trends; D015316:Genetic Therapy / Q000379:methods / Q000639:trends; D006816:Huntington Disease / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology*; D009410:Nerve Degeneration / Q000235:genetics / Q000378:metabolism*; D010455:Peptides / Q000037:antagonists & inhibitors* / Q000235:genetics / Q000378:metabolism; D019680:Trinucleotide Repeat Expansion / Q000235:genetics*
chemical_list: D010455:Peptides; C097188:polyglutamine