Neuronal circuitry regulates the response of Caenorhabditis elegans to misfolded proteins.

ABSTRACT: The consequence of chronic protein misfolding is the basis of many human diseases. To combat the deleterious effects of accumulated protein damage, all cells possess robust quality-control systems, specifically molecular chaperones and clearance machineries, that sense and respond to protein misfolding. However, for many protein conformational diseases, it is unclear why this quality-control system does not efficiently counter protein aggregation. Previous findings that the heat shock response in Caenorhabditis elegans is regulated by thermosensory neurons led us to consider whether neuronal activity could also be responsible for the inadequate response of an organism to chronic protein misfolding. Here we show, in animals expressing polyglutamine expansion proteins and mutant SOD-1(G93A)

SUBMITTER: Prahlad V 

PROVIDER: S-EPMC3161566 | biostudies-literature | 2011 Aug

REPOSITORIES: biostudies-literature