The pathogenic mechanisms of degenerative diseases of the nervous system are not well understood. Recent evidence suggests that proteins with a role in RNA synthesis, processing, function and degradation play a role in the mechanism of degenerative disorders affecting the motor neuron. However, most of these proteins also affect cellular processes other than RNA processing. Furthermore, many of the familial diseases are inherited dominantly, suggesting a gain-of-function as their pathogenic mechanism. This newly gained function could be unrelated to their normal role in the cell. Therefore, here we review some of the recent data linking RNA metabolism and motor neuron disorders, but also critically assess their relevance for our understanding of the mechanism of neurodegeneration.
Crown Copyright 2010. Published by Elsevier Ltd. All rights reserved.