Department of Biology, Brandeis University, Waltham, MA 02493, USA.
Stanley Center for Psychiatric Research, Broad Institute of Massachusetts Institute of Technology and Harvard University, Cambridge, MA, USA.
Neuron. 2020 Jun 3;106(5):769-777.e4. doi: 10.1016/j.neuron.2020.02.033. Epub 2020 Mar 20.
Mutations in Shank3 are strongly associated with autism spectrum disorders and neural circuit changes in several brain areas, but the cellular mechanisms that underlie these defects are not understood. Homeostatic forms of plasticity allow central circuits to maintain stable function during experience-dependent development, leading us to ask whether loss of Shank3 might impair homeostatic plasticity and circuit-level compensation to perturbations. We found that Shank3 loss in vitro abolished synaptic scaling and intrinsic homeostatic plasticity, deficits that could be rescued by treatment with lithium. Further, Shank3 knockout severely compromised the in vivo ability of visual cortical circuits to recover from perturbations to sensory drive. Finally, lithium treatment ameliorated a repetitive self-grooming phenotype in Shank3 knockout mice. These findings demonstrate that Shank3 loss severely impairs the ability of central circuits to harness homeostatic mechanisms to compensate for perturbations in drive, which, in turn, may render them more vulnerable to such perturbations.
Shank3 基因突变与自闭症谱系障碍和几个脑区的神经回路变化密切相关,但这些缺陷的细胞机制尚不清楚。 内稳态形式的可塑性使中枢回路能够在依赖经验的发育过程中维持稳定的功能,这促使我们提出 Shank3 的缺失是否会损害内稳态可塑性和对扰动的回路水平补偿。 我们发现,Shank3 的缺失在体外消除了突触缩放和内在的内稳态可塑性,而锂治疗可以挽救这些缺陷。 此外,Shank3 敲除严重损害了视觉皮层回路从感觉驱动扰动中恢复的体内能力。 最后,锂治疗改善了 Shank3 敲除小鼠的重复自我梳理表型。 这些发现表明,Shank3 的缺失严重损害了中枢回路利用内稳态机制来补偿驱动扰动的能力,这反过来又可能使它们更容易受到这种扰动的影响。