| Prepulse inhibition in patients with fragile X-associated tremor ataxia syndrome. | |
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MedLine Citation:
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PMID: 20961665 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Fragile X-associated tremor/ataxia syndrome (FXTAS) is a late onset neurodegenerative disorder that affects carriers of the fragile X premutation, typically after age 50. Common symptoms include intention tremor, ataxia, neuropathy, autonomic dysfunction, cognitive decline, and dementia. The objectives of this study were to determine if patients with FXTAS have altered prepulse inhibition (PPI; a measure of sensorimotor gating), and to study possible correlations between PPI, molecular status, and cognitive performance. A passive acoustic PPI paradigm was applied in 163 subjects; 121 carriers of the fragile X premutation, and 42 healthy controls. There were significant differences in PPI between premutation carriers with FXTAS and controls at PPI 60 ms, and at 120 ms. This effect was more prominent in the male FXTAS patients. There was a tendency to an impaired PPI in female premutation carriers at the 120 ms condition. There was a significant correlation between the PPI deficit and a higher CGG repeat number. The results show an impairment in sensorimotor gating processes in male carriers of the fragile X premutation, which is more prominent in patients with FXTAS. |
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Authors:
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Andrea Schneider; Elizabeth Ballinger; Alyssa Chavez; Flora Tassone; Randi J Hagerman; David Hessl |
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Publication Detail:
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Type: Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, P.H.S. Date: 2010-10-20 |
Journal Detail:
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Title: Neurobiology of aging Volume: 33 ISSN: 1558-1497 ISO Abbreviation: Neurobiol. Aging Publication Date: 2012 Jun |
Date Detail:
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Created Date: 2012-04-10 Completed Date: 2013-01-24 Revised Date: 2013-06-06 |
Medline Journal Info:
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Nlm Unique ID: 8100437 Medline TA: Neurobiol Aging Country: United States |
Other Details:
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Languages: eng Pagination: 1045-53 Citation Subset: IM |
Copyright Information:
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Copyright © 2012 Elsevier Inc. All rights reserved. |
Affiliation:
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Medical Investigation of Neurodevelopmental Disorders (M.I.N.D.) Institute, University of California at Davis, Sacramento, CA 95817, USA. andrea.schneider@ucdmc.ucdavis.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Acoustic Stimulation
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methods Aged Ataxia / epidemiology, genetics, physiopathology* Female Fragile X Mental Retardation Protein / genetics Fragile X Syndrome / epidemiology, genetics, physiopathology* Humans Male Middle Aged Neural Inhibition* / genetics Startle Reaction / genetics Tremor / epidemiology, genetics, physiopathology* |
| Grant Support | |
ID/Acronym/Agency:
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90DD05969/DD/NCBDD CDC HHS; AG032115/AG/NIA NIH HHS; AG032119/AG/NIA NIH HHS; HD03671/HD/NICHD NIH HHS; K23 MH077554-01A1/MH/NIMH NIH HHS; K23 MH077554-05/MH/NIMH NIH HHS; MH77554/MH/NIMH NIH HHS; R01 HD036071-13/HD/NICHD NIH HHS; R01 MH078041-05/MH/NIMH NIH HHS; RL1 AG032115-05/AG/NIA NIH HHS; RL1 AG032119-05/AG/NIA NIH HHS; UL1 DE019583-05/DE/NIDCR NIH HHS; UL1 RR024146/RR/NCRR NIH HHS; UL1 RR024146-01/RR/NCRR NIH HHS; UL1 RR024146-05/RR/NCRR NIH HHS; UL1DE019583/DE/NIDCR NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/FMR1 protein, human; 139135-51-6/Fragile X Mental Retardation Protein |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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