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Updated: Mar 27, 2026

Dissecting Cell-Autonomous Function of Fragile X Mental Retardation Protein in an Auditory Circuit by In Ovo Electroporation
Published on: July 6, 2022
HPA axis function predicts development of working memory in boys with FXS
Jessica F Scherr1, Laura J Hahn1, Stephen R Hooper2
1Department of Psychology, 1512 Pendleton Street, University of South Carolina, Columbia, SC 29208, USA.
Boys with fragile X syndrome (FXS) show verbal working memory deficits, particularly in memory for words, worsened by stress (cortisol). Cognitive development in FXS is influenced by biological and cognitive interactions over time.
Area of Science:
- Neuroscience
- Developmental Psychology
- Genetics
Background:
- Fragile X syndrome (FXS) is a genetic disorder associated with cognitive impairments.
- Understanding the developmental trajectory of cognitive functions, like verbal working memory, in FXS is crucial.
- The role of physiological stress markers, such as cortisol, in cognitive development within FXS is not fully understood.
Purpose of the Study:
- To examine longitudinal changes in verbal working memory in boys with FXS compared to typically developing (TD) controls.
- To investigate the relationship between cortisol levels and verbal working memory performance over time in boys with FXS.
- To elucidate the interplay between biological stress mechanisms and cognitive development in FXS.
Main Methods:
- Longitudinal study design with assessments over a 2-year period (1-3 assessments per participant).
- Inclusion of boys with FXS and nonverbal mental-age (NVMA) matched TD boys.
- Utilized two verbal working memory tasks of varying complexity (memory for words, auditory working memory) and salivary cortisol measurements.
Main Results:
- Boys with FXS demonstrated specific longitudinal deficits in memory for words compared to TD controls.
- Elevated baseline cortisol exacerbated verbal working memory deficits in boys with FXS on the memory for words task.
- While both groups showed similar growth rates in auditory working memory, higher baseline cortisol was associated with lower performance in boys with FXS.
Conclusions:
- Verbal working memory development in boys with FXS is characterized by specific deficits, particularly in tasks requiring word recall.
- Cortisol, a marker of physiological stress, significantly impacts verbal working memory performance in boys with FXS.
- Investigating the dynamic interactions between biological factors (cortisol) and cognitive development (verbal working memory) is essential for understanding FXS.
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