Another degenerative disease is caused by aberrant RNA, according to two studies published this week in Neuron. The results, which add a second disease to the list of conditions that stem from mutant RNA, suggest that sequestration of protein by mutant RNA may be a more general disease mechanism than previously suspected.Researchers studying myotonic dystrophy (DM), a neuromuscular degenerative condition, found a decade ago that DM was mediated by an elongated repeating sequence of three or four nucleotides in untranslated RNA that bound up proteins responsible for RNA processing. The current work confirms that in Fragile X Tremor/Ataxia Syndrome (FXTAS), also a neuromuscular degenerative disease marked by trinucleotide repeats, RNA pathogenesis is also to blame."It's looking more and more like the story with DM," said the University of Minnesota's Harry Orr, who cowrote the papers' accompanying commentary. "The point is you get the disease because of...
Peng JinstudyDrosophilaStephen WarrenLaura RanumMaurice Swanson
mail@the-scientist.comalpha Binds to rCGG Repeats and Modulates Repeat-Mediated Neurodegeneration in a Drosophila Model of Fragile X Tremor/Ataxia Syndrome," Neuron, August 16, 2007.
O.A. Sofola et al., "RNA-binding proteins hnRNP A2/B1 and CUGBP1 suppress fragile X CGG premutation repeat-induced neurodegeneration in a drosophila model of FXTAS," Neuron, August 16, 2007.
S. Bunk, "The dark side of RNA," The Scientist, November 12, 2001.
J.B. Weitzman, "Myotonic expansion," The Scientist, August 6, 2001.
Harry Orr
M.S. Swanson and H.T. Orr, "Fragile X Tremor/Ataxia Syndrome: Blame the messenger!," Neuron, August 16, 2007.
Peng Jin Jin et al., "RNA-mediated neurodegeneration caused by the fragile X premutation rCGG repeats in Drosophila," Neuron, August 28, 2003. Warren Ranum Swanson

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