Department of Genetics, Albert Einstein College of Medicine, Bronx, New York, United States of America.
Department of Neurology, Albert Einstein College of Medicine, Bronx, New York, United States of America.
PLoS One. 2014 Apr 15;9(4):e94968. doi: 10.1371/journal.pone.0094968. eCollection 2014.
Schizophrenia (SZ) and autism spectrum disorders (ASD) are highly heritable neuropsychiatric disorders, although environmental factors, such as maternal immune activation (MIA), play a role as well. Cytokines mediate the effects of MIA on neurogenesis and behavior in animal models. However, MIA stimulators can also induce a febrile reaction, which could have independent effects on neurogenesis through heat shock (HS)-regulated cellular stress pathways. However, this has not been well-studied. To help understand the role of fever in MIA, we used a recently described model of human brain development in which induced pluripotent stem cells (iPSCs) differentiate into 3-dimensional neuronal aggregates that resemble a first trimester telencephalon. RNA-seq was carried out on aggregates that were heat shocked at 39°C for 24 hours, along with their control partners maintained at 37°C. 186 genes showed significant differences in expression following HS (p<0.05), including known HS-inducible genes, as expected, as well as those coding for NGFR and a number of SZ and ASD candidates, including SMARCA2, DPP10, ARNT2, AHI1 and ZNF804A. The degree to which the expression of these genes decrease or increase during HS is similar to that found in copy loss and copy gain copy number variants (CNVs), although the effects of HS are likely to be transient. The dramatic effect on the expression of some SZ and ASD genes places HS, and perhaps other cellular stressors, into a common conceptual framework with disease-causing genetic variants. The findings also suggest that some candidate genes that are assumed to have a relatively limited impact on SZ and ASD pathogenesis based on a small number of positive genetic findings, such as SMARCA2 and ARNT2, may in fact have a much more substantial role in these disorders - as targets of common environmental stressors.
精神分裂症 (SZ) 和自闭症谱系障碍 (ASD) 是高度遗传性的神经精神疾病,尽管环境因素,如母体免疫激活 (MIA),也起作用。细胞因子在动物模型中介导 MIA 对神经发生和行为的影响。然而,MIA 刺激物也会引起发热反应,这可能通过热休克 (HS) 调节的细胞应激途径对神经发生产生独立的影响。然而,这一点还没有得到很好的研究。为了帮助理解发热在 MIA 中的作用,我们使用了一种最近描述的人类大脑发育模型,其中诱导多能干细胞 (iPSC) 分化为类似于头三个月端脑的 3 维神经元聚集体。将聚集体在 39°C 下热休克 24 小时,并与在 37°C 下保持的对照物一起进行 RNA-seq。186 个基因在 HS 后表达差异显著 (p<0.05),包括已知的 HS 诱导基因,如预期的那样,以及编码 NGFR 和一些 SZ 和 ASD 候选基因的基因,包括 SMARCA2、DPP10、ARNT2、AHI1 和 ZNF804A。这些基因在 HS 期间表达减少或增加的程度与在拷贝丢失和拷贝增益拷贝数变异 (CNV) 中发现的相似,尽管 HS 的影响可能是短暂的。一些 SZ 和 ASD 基因的表达受到如此显著的影响,这使得 HS,以及其他可能的细胞应激源,与致病遗传变异处于一个共同的概念框架中。这些发现还表明,一些候选基因,如 SMARCA2 和 ARNT2,根据少数阳性遗传发现,假设它们对 SZ 和 ASD 发病机制的影响相对有限,实际上可能在这些疾病中发挥更为重要的作用——作为常见环境应激源的靶点。