Article
RNA-mediated neurodegeneration in repeat expansion disorders.
Annals of neurology - 1 Mar 2010
Todd Peter K, Paulson Henry L
Abstract excerpt
Most neurodegenerative disorders are thought to result primarily from the accumulation of misfolded proteins, which interfere with protein homeostasis in neurons. For a subset of diseases, however, noncoding regions of RNAs assume a primary toxic gain-of-function, leading to degeneration in many tissues, including the nervous system. Here we review a series of proposed mechanisms by which noncoding repeat...
Topics
- Fragile X Syndrome
- Genetic Predisposition to Disease
- Heredodegenerative Disorders, Nervous System
- Humans
- Myotonic Dystrophy
- RNA
- RNA Splice Sites
- RNA, Antisense
- RNA, Messenger
- RNA, Untranslated
- Trinucleotide Repeat Expansion