Taylor Avigail, Steinberg Julia, Webber Caleb
MRC Functional Genomics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford, United Kingdom.
Am J Med Genet B Neuropsychiatr Genet. 2015 Mar;168B(2):97-107. doi: 10.1002/ajmg.b.32285. Epub 2015 Feb 5.
Attention deficit/hyperactivity disorder (ADHD) is a childhood onset disorder, prevalent in 5.3% of children and 1-4% of adults. ADHD is highly heritable, with a burden of large (>500 Kb) copy number variants (CNVs) identified among individuals with ADHD. However, how such CNVs exert their effects is poorly understood. We examined the genes affected by 71 large, rare, and predominantly inherited CNVs identified among 902 individuals with ADHD. We applied both mouse-knockout functional enrichment analyses, exploiting behavioral phenotypes arising from the determined disruption of 1:1 mouse orthologues, and human brain-specific spatio-temporal expression data to uncover molecular pathways common among genes contributing to enriched phenotypes. Twenty-two percent of genes duplicated in individuals with ADHD that had mouse phenotypic information were associated with abnormal learning/memory/conditioning ("l/m/c") phenotypes. Although not observed in a second ADHD-cohort, we identified a similar enrichment among genes duplicated by eight de novo CNVs present in eight individuals with Hyperactivity and/or Short attention span ("Hyperactivity/SAS", the ontologically-derived phenotypic components of ADHD). In the brain, genes duplicated in patients with ADHD and Hyperactivity/SAS and whose orthologues' disruption yields l/m/c phenotypes in mouse ("candidate-genes"), were co-expressed with one another and with genes whose orthologues' mouse models exhibit hyperactivity. Moreover, genes associated with hyperactivity in the mouse were significantly more co-expressed with ADHD candidate-genes than with similarly identified genes from individuals with intellectual disability. Our findings support an etiology for ADHD distinct from intellectual disability, and mechanistically related to genes associated with hyperactivity phenotypes in other mammalian species.
注意缺陷多动障碍(ADHD)是一种起病于儿童期的疾病,在5.3%的儿童和1 - 4%的成年人中普遍存在。ADHD具有高度遗传性,在ADHD患者中已鉴定出存在大量(>500 Kb)拷贝数变异(CNV)。然而,此类CNV如何发挥作用却知之甚少。我们研究了在902例ADHD患者中鉴定出的71个大型、罕见且主要为遗传性的CNV所影响的基因。我们应用了小鼠基因敲除功能富集分析,利用由确定破坏1:1小鼠直系同源基因所产生的行为表型,以及人类大脑特异性时空表达数据,来揭示在导致富集表型的基因中常见的分子途径。在有小鼠表型信息的ADHD患者中,22%的重复基因与异常学习/记忆/条件反射(“l/m/c”)表型相关。尽管在第二个ADHD队列中未观察到,但我们在8例多动和/或注意力持续时间短(“多动/注意力不集中,SAS”,ADHD在本体论上衍生的表型成分)患者中存在的8个新发CNV所重复的基因中发现了类似的富集现象。在大脑中,ADHD和多动/注意力不集中,SAS患者中重复且其直系同源基因的破坏在小鼠中产生l/m/c表型的基因(“候选基因”),彼此之间以及与直系同源基因的小鼠模型表现出多动的基因共同表达。此外,与小鼠多动相关的基因与ADHD候选基因的共表达显著多于与来自智力残疾个体中类似鉴定基因的共表达。我们的研究结果支持ADHD的病因与智力残疾不同,且在机制上与其他哺乳动物物种中与多动表型相关的基因有关。