RNA gains a new function: A mediator of neurodegeneration

Trends in Neurosciences

Volumn 27, Issue 5, 2004, Pages 233-234

  Orr, Harry T ^a  

^a UNIVERSITY OF MINNESOTA MEDICAL SCHOOL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CARRIER PROTEIN; FRAGILE X MENTAL RETARDATION PROTEIN; HEAT SHOCK PROTEIN 70; POLYGLUTAMINE; RNA; UBIQUITIN;

5' UNTRANSLATED REGION; ALLELE; ATAXIA; CELL NUCLEUS INCLUSION BODY; DEGENERATIVE DISEASE; DROSOPHILA; GENE DOSAGE; GENE EXPRESSION; GENE MUTATION; GENETIC TRANSCRIPTION; HETEROZYGOTE; HUMAN; NERVE DEGENERATION; NONHUMAN; PRIORITY JOURNAL; PROTEIN FUNCTION; PROTEIN RNA BINDING; SHORT SURVEY; TREMOR; UBIQUITINATION;

ANIMALS; FRAGILE X MENTAL RETARDATION PROTEIN; HSP70 HEAT-SHOCK PROTEINS; HUMANS; INCLUSION BODIES; MALE; NERVE DEGENERATION; NERVE TISSUE PROTEINS; NEURODEGENERATIVE DISEASES; RNA; RNA-BINDING PROTEINS; UBIQUITIN;

EID: 1942532863     PISSN: 01662236     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.tins.2004.03.003     Document Type: Short Survey

References (14)

1. Verkerk A.J.M.H., et al. Identification of a gene (FMR-1) containing a CGG repeat coincident with a breakpoint cluster region exhibiting length variation in fragile X syndrome. Cell. 65:1991;905-914.
2. Gedeon A.K., et al. Fragile X syndrome without CGG amplification has an FMR1 phenotype. Nat. Genet. 1:1992;341-344.
3. Wohrle D., et al. A microdeletion of less than 250kb, including the proximal part of the FMR-1 gene and the fragile-X site, in a male with the clinical phenotype of fragile-X syndrome. Am. J. Hum. Genet. 51:1992;299-306.
4. Sherman S.L., et al. Further segregation analysis of the fragile X syndrome with special reference to transmitting males. Hum. Genet. 69:1985;289-299.
5. Hagerman R.J., et al. intention tremor, parkinsonism, and generalized brain atrophy in male carriers of fragile X. Neurology. 57:2001;127-130.
6. Greco C.M., et al. Neuronal intranuclear inclusions in a new cerebellar tremor/ataxia syndrome among fragile X carriers. Brain. 125:2002;1760-1771.
7. Tassone F., et al. Elevated levels of FMR1 mRNA in carrier males: a new mechanism of involvement in the fragile-X syndrome. Am. J. Hum. Genet. 66:2000;6-15.
8. Kenneson A., et al. Reduced FMRP and increased transcription is proportionally associated with CGG repeat number in intermediate-length and premutation carriers. Hum. Mol. Genet. 10:2001;1449-1454.
9. Ranum L.P.W., Day J.W. Dominantly inherited, non-coding microsatellite expansion disorders. Curr. Opin. Genet. Dev. 12:2002;266-271.
10. Zoghbi H.Y., Botas J. Mouse and fly models of neurodegeneration. Trends Genet. 18:2002;463-471.
11. Jin P., et al. RNA-mediated neurodegeneration caused by the fragile X premutation rCGG repeats in Drosophila. Neuron. 39:2003;739-747.
12. Zoghbi H.Y., Orr H.T. Glutamine repeats and neurodegeneration. Annu. Rev. Neurosci. 23:2000;217-247.
13. Proudfoot N.J., et al. Integrating mRNA processing with transcription. Cell. 108:2002;501-512.
14. Weissman A.M. Themes and variations on ubiquitylation. Nat. Rev. Mol. Cell Biol. 2:2001;169-178.