Journal
NEUROLOGY
Volume 57, Issue 1, Pages 127-130Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1212/WNL.57.1.127
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Funding
- NCRR NIH HHS [MO1 RR00069] Funding Source: Medline
- NICHD NIH HHS [HD36071] Funding Source: Medline
- PHS HHS [MCJ-089413] Funding Source: Medline
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The authors report five elderly men with the fragile X premutation who had a progressive action tremor associated with executive function deficits and generalized brain atrophy. These individuals had elevated fragile X mental retardation 1 gene (FMR1) messenger RNA and normal or borderline levels of FMR1 protein. The authors propose that elevations of FMR1 messenger RNA may be causative for a neurodegenerative syndrome in a subgroup of elderly men with the FMR1 premutation.
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