Genes · 2020
7q35 Microdeletion and 15q13.3 and Xp22.33 Microduplications in a Patient with Severe Myoclonic Epilepsy, Microcephaly, Dysmorphisms, Severe Psychomotor Delay and Intellectual Disability.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Paduano Francesco, Colao Emma, Loddo Sara, Orlando Valeria, Trapasso Francesco, Novelli Antonio, Perrotti Nicola, Iuliano Rodolfo
Аннотация
Copy number variations (CNVs) play a key role in the pathogenesis of several diseases, including a wide range of neurodevelopmental disorders. Here, we describe the detection of three CNVs simultaneously in a female patient with evidence of severe myoclonic epilepsy, microcephaly, hypertelorism, dimorphisms as well as severe psychomotor delay and intellectual disability. Array-CGH analysis revealed a ∼240 kb microdeletion at the 7q35 inherited from her father, a ∼538 kb microduplication at the 15q13.3 region and a ∼178 kb microduplication at Xp22.33 region, both transmitted from her mother. The microdeletion in 7q35 was included within an intragenic region of the contactin associated protein-like 2 (CNTNAP2) gene, whereas the microduplications at 15q13.3 and Xp22.33 involved the cholinergic receptor nicotinic alpha 7 subunit (CHRNA7) and the cytokine receptor-like factor 2 (CRLF2) genes, respectively. Here, we describe a female patient harbouring three CNVs whose additive contribution could be responsible for her clinical phenotypes.
Предметные рубрики MeSH
AdultChromosomes, Human, Pair 15Chromosomes, Human, Pair 7Chromosomes, Human, XConsanguinityDNA Copy Number VariationsEpilepsies, MyoclonicFemaleGene DuplicationGenetic Association StudiesHumansMembrane ProteinsMicrocephalyNerve Tissue ProteinsNeurodevelopmental DisordersPedigreeReceptors, CytokineSequence DeletionTissue Array Analysisalpha7 Nicotinic Acetylcholine Receptor