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Adult Female Fragile X Premutation Carriers Exhibit Age- and CGG Repeat Length-Related Impairments on an Attentionally Based Enumeration Task
The high frequency of the fragile X premutation in the general population and its emerging neurocognitive implications highlight the need to investigate the effects of the premutation on lifespan cognitive development. Until recently, cognitive function in fragile X premutation carriers (fXPCs) was...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Research Foundation
2011
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3139190/ https://www.ncbi.nlm.nih.gov/pubmed/21808616 http://dx.doi.org/10.3389/fnhum.2011.00063 |
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author | Goodrich-Hunsaker, Naomi J. Wong, Ling M. McLennan, Yingratana Tassone, Flora Harvey, Danielle Rivera, Susan M. Simon, Tony J. |
author_facet | Goodrich-Hunsaker, Naomi J. Wong, Ling M. McLennan, Yingratana Tassone, Flora Harvey, Danielle Rivera, Susan M. Simon, Tony J. |
author_sort | Goodrich-Hunsaker, Naomi J. |
collection | PubMed |
description | The high frequency of the fragile X premutation in the general population and its emerging neurocognitive implications highlight the need to investigate the effects of the premutation on lifespan cognitive development. Until recently, cognitive function in fragile X premutation carriers (fXPCs) was presumed to be unaffected by the mutation. Although as a group fXPCs did not differ from healthy controls (HCs), we show that young adult female fXPCs show subtle age- and significant fragile X mental retardation 1 (FMR1) gene mutation-modulated cognitive function as tested by a basic numerical enumeration task. These results indicate that older women with the premutation and fXPCs with greater CGG repeat lengths were at higher risk for difficulties in the deployment of volitional attention required to count 5–8 items, but spared performance when spatial shifts of attention were minimized to subitize a few (1–3). Results from the current study add to a growing body of evidence that suggests the premutation allele is associated with a subtle phenotype and implies that the cognitive demands necessary for counting are less effectively deployed in female fXPCs compared to HCs. |
format | Online Article Text |
id | pubmed-3139190 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2011 |
publisher | Frontiers Research Foundation |
record_format | MEDLINE/PubMed |
spelling | pubmed-31391902011-08-01 Adult Female Fragile X Premutation Carriers Exhibit Age- and CGG Repeat Length-Related Impairments on an Attentionally Based Enumeration Task Goodrich-Hunsaker, Naomi J. Wong, Ling M. McLennan, Yingratana Tassone, Flora Harvey, Danielle Rivera, Susan M. Simon, Tony J. Front Hum Neurosci Neuroscience The high frequency of the fragile X premutation in the general population and its emerging neurocognitive implications highlight the need to investigate the effects of the premutation on lifespan cognitive development. Until recently, cognitive function in fragile X premutation carriers (fXPCs) was presumed to be unaffected by the mutation. Although as a group fXPCs did not differ from healthy controls (HCs), we show that young adult female fXPCs show subtle age- and significant fragile X mental retardation 1 (FMR1) gene mutation-modulated cognitive function as tested by a basic numerical enumeration task. These results indicate that older women with the premutation and fXPCs with greater CGG repeat lengths were at higher risk for difficulties in the deployment of volitional attention required to count 5–8 items, but spared performance when spatial shifts of attention were minimized to subitize a few (1–3). Results from the current study add to a growing body of evidence that suggests the premutation allele is associated with a subtle phenotype and implies that the cognitive demands necessary for counting are less effectively deployed in female fXPCs compared to HCs. Frontiers Research Foundation 2011-07-14 /pmc/articles/PMC3139190/ /pubmed/21808616 http://dx.doi.org/10.3389/fnhum.2011.00063 Text en Copyright © 2011 Goodrich-Hunsaker, Wong, McLennan, Tassone, Harvey, Rivera and Simon. http://www.frontiersin.org/licenseagreement This is an open-access article subject to a non-exclusive license between the authors and Frontiers Media SA, which permits use, distribution and reproduction in other forums, provided the original authors and source are credited and other Frontiers conditions are complied with. |
spellingShingle | Neuroscience Goodrich-Hunsaker, Naomi J. Wong, Ling M. McLennan, Yingratana Tassone, Flora Harvey, Danielle Rivera, Susan M. Simon, Tony J. Adult Female Fragile X Premutation Carriers Exhibit Age- and CGG Repeat Length-Related Impairments on an Attentionally Based Enumeration Task |
title | Adult Female Fragile X Premutation Carriers Exhibit Age- and CGG Repeat Length-Related Impairments on an Attentionally Based Enumeration Task |
title_full | Adult Female Fragile X Premutation Carriers Exhibit Age- and CGG Repeat Length-Related Impairments on an Attentionally Based Enumeration Task |
title_fullStr | Adult Female Fragile X Premutation Carriers Exhibit Age- and CGG Repeat Length-Related Impairments on an Attentionally Based Enumeration Task |
title_full_unstemmed | Adult Female Fragile X Premutation Carriers Exhibit Age- and CGG Repeat Length-Related Impairments on an Attentionally Based Enumeration Task |
title_short | Adult Female Fragile X Premutation Carriers Exhibit Age- and CGG Repeat Length-Related Impairments on an Attentionally Based Enumeration Task |
title_sort | adult female fragile x premutation carriers exhibit age- and cgg repeat length-related impairments on an attentionally based enumeration task |
topic | Neuroscience |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3139190/ https://www.ncbi.nlm.nih.gov/pubmed/21808616 http://dx.doi.org/10.3389/fnhum.2011.00063 |
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