TY - JOUR
T1 - RNA gains a new function
T2 - A mediator of neurodegeneration
AU - Orr, Harry T.
N1 - Copyright:
Copyright 2008 Elsevier B.V., All rights reserved.
PY - 2004/5
Y1 - 2004/5
N2 - Over the past couple of years, it has become evident that some males carrying the fragile X mental retardation (FMR1) premutation, typically viewed to be normal, in fact manifest a neurodegenerative disease with ataxia and tremor. FMR1 premutation alleles uniquely produce FMR1 transcripts with an elongated CGG repeat, leading to the hypothesis that premutant transcripts cause the neurodegenerative disease in carriers. Recently Jin et al. demonstrated, in Drosophila, that FMR1 premutation RNA causes neurodegeneration. These data show RNA can induce neurodegeneration and provide strong evidence that FMR1 RNA mediates the neurodegeneration in human premutation carriers.
AB - Over the past couple of years, it has become evident that some males carrying the fragile X mental retardation (FMR1) premutation, typically viewed to be normal, in fact manifest a neurodegenerative disease with ataxia and tremor. FMR1 premutation alleles uniquely produce FMR1 transcripts with an elongated CGG repeat, leading to the hypothesis that premutant transcripts cause the neurodegenerative disease in carriers. Recently Jin et al. demonstrated, in Drosophila, that FMR1 premutation RNA causes neurodegeneration. These data show RNA can induce neurodegeneration and provide strong evidence that FMR1 RNA mediates the neurodegeneration in human premutation carriers.
UR - http://www.scopus.com/inward/record.url?scp=1942532863&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=1942532863&partnerID=8YFLogxK
U2 - 10.1016/j.tins.2004.03.003
DO - 10.1016/j.tins.2004.03.003
M3 - Short survey
C2 - 15111000
AN - SCOPUS:1942532863
SN - 0166-2236
VL - 27
SP - 233
EP - 234
JO - Trends in Neurosciences
JF - Trends in Neurosciences
IS - 5
ER -