European journal of human genetics : EJHG · 2011
Translocation breakpoint at 7q31 associated with tics: further evidence for IMMP2L as a candidate gene for Tourette syndrome.
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Patel Chirag, Cooper-Charles Lisa, McMullan Dominic J, Walker Judith M, Davison Val, Morton Jenny
Аннотация
Gilles de la Tourette syndrome is a complex neuropsychiatric disorder with a strong genetic basis. We identified a male patient with Tourette syndrome-like tics and an apparently balanced de novo translocation [46,XY,t(2;7)(p24.2;q31)]. Further analysis using array comparative genomic hybridisation (CGH) revealed a cryptic deletion at 7q31.1-7q31.2. Breakpoints disrupting this region have been reported in one isolated and one familial case of Tourette syndrome. In our case, IMMP2L, a gene coding for a human homologue of the yeast inner mitochondrial membrane peptidase subunit 2, was disrupted by the breakpoint on 7q31.1, with deletion of exons 1-3 of the gene. The IMMP2L gene has previously been proposed as a candidate gene for Tourette syndrome, and our case provides further evidence of its possible role in the pathogenesis. The deleted region (7q31.1-7q31.2) of 7.2 Mb of genomic DNA also encompasses numerous genes, including FOXP2, associated with verbal dyspraxia, and the CFTR gene.
Предметные рубрики MeSH
ApraxiasChromosome BreakpointsChromosomes, Human, Pair 7Comparative Genomic HybridizationCystic Fibrosis Transmembrane Conductance RegulatorDNAEndopeptidasesExonsForkhead Transcription FactorsHumansIn Situ Hybridization, FluorescenceMaleOligonucleotide Array Sequence AnalysisPedigreeSequence DeletionTicsTourette SyndromeTranslocation, Genetic