Fragile X syndrome and associated disorders: Clinical aspects and pathology.

Neurobiol Dis

Department of Pediatrics, University of California Davis School of Medicine, Sacramento, CA, USA; Medical Investigation of Neurodevelopmental Disorders (MIND) Institute, University of California Davis, Sacramento, CA, USA. Electronic address:

Published: March 2020

This review aims to assemble many years of research and clinical experience in the fields of neurodevelopment and neuroscience to present an up-to-date understanding of the clinical presentation, molecular and brain pathology associated with Fragile X syndrome, a neurodevelopmental condition that develops with the full mutation of the FMR1 gene, located in the q27.3 loci of the X chromosome, and Fragile X-associated tremor/ataxia syndrome a neurodegenerative disease experienced by aging premutation carriers of the FMR1 gene. It is important to understand that these two syndromes have a very distinct clinical and pathological presentation while sharing the same origin: the mutation of the FMR1 gene; revealing the complexity of expansion genetics.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7027994PMC
http://dx.doi.org/10.1016/j.nbd.2020.104740DOI Listing

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