U.O. e Cattedra di Neuropsichiatria Infantile, Istituto G. Gaslini, Genova, Italy.
Gene. 2013 Dec 15;532(2):294-6. doi: 10.1016/j.gene.2013.09.073. Epub 2013 Sep 28.
Mutations in neuronal voltage-gated sodium channel genes SCN1A, SCN2A, and SCN3A may play an important role in the etiology of neurological diseases and psychiatric disorders, besides various types of epilepsy. Here we describe a 3-year-old boy with autistic features, language delay, microcephaly and no history of seizures. Array-CGH analysis revealed an interstitial deletion of ~291.9kB at band 2q24.3 disrupting the entire SCN2A gene and part of SCN3A. We discuss the effects of haploinsufficiency of SCN2A and SCN3A on the genetic basis of neurodevelopmental and neurobehavioral disorders and we propose that this haploinsufficiency may be associated not only with epilepsy, but also with autistic features.
神经元电压门控钠离子通道基因 SCN1A、SCN2A 和 SCN3A 的突变除了各种类型的癫痫外,可能在神经和精神疾病的发病机制中发挥重要作用。在这里,我们描述了一个有自闭症特征、语言发育迟缓、小头畸形且无癫痫发作史的 3 岁男孩。阵列-CGH 分析显示,在 2q24.3 带区存在约 291.9kb 的片段缺失,破坏了整个 SCN2A 基因和部分 SCN3A 基因。我们讨论了 SCN2A 和 SCN3A 的单倍不足对神经发育和神经行为障碍的遗传基础的影响,并提出这种单倍不足不仅与癫痫有关,还与自闭症特征有关。