Patricia Maciel

Ana João Rodrigues

Several neurodegenerative diseases are linked to the production of mutated proteins, which accumulate in the brain of patients and are believed to lead to the neurons death characteristic of these disorders. Nevertheless, despite this shared mechanism, each of these diseases is unique, as are the brain areas affected, and to understand them is crucial to identify the biological function of the mutated protein behind the disease, something not always easy. But now, an elegant study just published in the advance online edition of the Faseb(1) journal shows how the study of a simpler genetic model - the worm Caenorhabditis elegans (C.elegans) – was able to give insight into the function of the human protein ataxin-3, which when mutated is linked to Machado-Joseph disease (MJD), a fatal genetic neurodegenerative disorder.

*"This article is the result of a project for the divulgation of portuguese science" http://www.cienciahoje.pt/1455"