Abstract
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.
| Original language | English (US) |
|---|---|
| Article number | 63 |
| Journal | Frontiers in Human Neuroscience |
| Issue number | JULY |
| DOIs | |
| State | Published - 2011 |
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Keywords
- X-linked genetic disease
ASJC Scopus subject areas
- Psychiatry and Mental health
- Neurology
- Biological Psychiatry
- Behavioral Neuroscience
- Neuropsychology and Physiological Psychology
Cite this
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 J; Rivera, Susan M.; Simon, Tony J.
In: Frontiers in Human Neuroscience, No. JULY, 63, 2011.Research output: Contribution to journal › Article
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TY - JOUR
T1 - Adult female fragile X premutation carriers exhibit age- and CGG repeat length-related impairments on an attentionally based enumeration task
AU - Goodrich-Hunsaker, Naomi J.
AU - Wong, Ling M.
AU - McLennan, Yingratana
AU - Tassone, Flora
AU - Harvey, Danielle J
AU - Rivera, Susan M.
AU - Simon, Tony J
PY - 2011
Y1 - 2011
N2 - 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.
AB - 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.
KW - X-linked genetic disease
UR - http://www.scopus.com/inward/record.url?scp=82955198526&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=82955198526&partnerID=8YFLogxK
U2 - 10.3389/fnhum.2011.00063
DO - 10.3389/fnhum.2011.00063
M3 - Article
C2 - 21808616
AN - SCOPUS:82955198526
JO - Hematology / the Education Program of the American Society of Hematology. American Society of Hematology. Education Program
JF - Hematology / the Education Program of the American Society of Hematology. American Society of Hematology. Education Program
SN - 1662-5161
IS - JULY
M1 - 63
ER -