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Frontostriatal deficits in fragile X syndrome: relation to FMR1 gene expression.


ABSTRACT: Fragile X syndrome (fraX) is the most common known cause of inherited developmental disability. fraX is associated with a CGG expansion in the FMR1 gene on the long arm of the X chromosome. Behavioral deficits, including problems with impulse control and distractibility, are common in fraX. We used functional brain imaging with a Go/NoGo task to examine the neural substrates of response inhibition in females with fraX (ages 10-22) and age- and gender-matched typically developing subjects. Although subjects with fraX had significantly lower IQ scores, as a group their performance on the Go/NoGo task was equivalent to that of the typically developing group. However, females with fraX showed abnormal activation patterns in several cortical and subcortical regions, with significantly reduced a

SUBMITTER: Menon V 

PROVIDER: S-EPMC373511 | biostudies-literature | 2004 Mar

REPOSITORIES: biostudies-literature