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Official websites use. Share sensitive information only on official, secure websites. Analysis of single DNA molecules with nanopore sequencing and molecular combing show that expansions range from 3. However, we observe considerable variability in expansion length and structure, supporting the existence of multiple expansion configurations in blood cells and fibroblasts of the same individual.

Subject terms: Genomic instability, Epilepsy, Neurodegenerative diseases, Neurological disorders. Here, Florian et al. FAME is an autosomal dominant, very slowly progressive condition characterized by cortical tremor affecting mainly the hands, frequently associated with generalized myoclonic and sometimes tonic-clonic seizures, and, more rarely, focal seizures 1 β€” 3.

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Several different chromosome loci, identified through linkage, at 2pq11, 3qq28, 5p15, and 8q24, have been reported 4 β€” 7 but the genetic variants underlying the disorder have remained elusive for 20 years despite extensive sequencing of genes contained in these intervals. SAMD12 pentanucleotide repeat expansions are associated with a specific haplotype originating from a founder effect in Asia 8 , Sequencing of all exons in the linked interval by next generation sequencing had excluded the existence of pathogenic coding variants.

GluLys in CTNND2 , which segregated in all affected family members but one, who was considered a possible phenocopy 13 , Individuals with ID numbers in red are carriers of the expansions.

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Individuals with ID numbers underlined have been included in whole-genome sequencing analyses. Individuals with stars have been included in RNA-seq analyses.

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