| Literature DB >> 31927143 |
Maria Jimena Salcedo-Arellano1, Brett Dufour2, Yingratana McLennan3, Veronica Martinez-Cerdeno4, Randi Hagerman5.
Abstract
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.Entities:
Keywords: FMR1 gene; FXTAS; Fragile X syndrome; Fragile X-associated tremor/ataxia syndrome; Neuropathology
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Year: 2020 PMID: 31927143 PMCID: PMC7027994 DOI: 10.1016/j.nbd.2020.104740
Source DB: PubMed Journal: Neurobiol Dis ISSN: 0969-9961 Impact factor: 7.046