Neuroelectronics Research Flanders (NERF), KU Leuven, Kapeldreef 75, 3001 Leuven, Belgium; VIB Center for the Biology of Disease, KU Leuven, Herestraat 49, 3000 Leuven, Belgium; Center for Human Genetics, KU Leuven, Herestraat 49, 3000 Leuven, Belgium.
VIB Center for the Biology of Disease, KU Leuven, Herestraat 49, 3000 Leuven, Belgium; Center for Human Genetics, KU Leuven, Herestraat 49, 3000 Leuven, Belgium.
Curr Biol. 2017 Apr 24;27(8):1111-1123. doi: 10.1016/j.cub.2017.02.065. Epub 2017 Mar 30.
Fragile X syndrome (FXS) patients present neuronal alterations that lead to severe intellectual disability, but the underlying neuronal circuit mechanisms are poorly understood. An emerging hypothesis postulates that reduced GABAergic inhibition of excitatory neurons is a key component in the pathophysiology of FXS. Here, we directly test this idea in a FXS Drosophila model. We show that FXS flies exhibit strongly impaired olfactory behaviors. In line with this, olfactory representations are less odor specific due to broader response tuning of excitatory projection neurons. We find that impaired inhibitory interactions underlie reduced specificity in olfactory computations. Finally, we show that defective lateral inhibition across projection neurons is caused by weaker inhibition from GABAergic interneurons. We provide direct evidence that deficient inhibition impairs sensory computations and behavior in an in vivo model of FXS. Together with evidence of impaired inhibition in autism and Rett syndrome, these findings suggest a potentially general mechanism for intellectual disability.
脆性 X 综合征 (FXS) 患者存在神经元改变,导致严重的智力障碍,但神经元回路的潜在机制仍不清楚。一个新兴的假说假设兴奋性神经元 GABA 能抑制的减弱是 FXS 病理生理学的一个关键组成部分。在这里,我们在 FXS 果蝇模型中直接检验了这一观点。我们发现 FXS 果蝇表现出强烈受损的嗅觉行为。与此一致的是,由于兴奋性投射神经元的反应调谐更广泛,嗅觉表现出的气味特异性降低。我们发现,抑制性相互作用的受损是由于 GABA 能中间神经元的抑制作用减弱所致,从而导致嗅觉计算的特异性降低。最后,我们发现投射神经元之间的缺陷侧抑制是由 GABA 能中间神经元的抑制作用减弱引起的。我们提供了直接的证据,表明在 FXS 的体内模型中,抑制作用的缺失会损害感觉计算和行为。结合自闭症和雷特综合征中抑制作用受损的证据,这些发现表明智力障碍可能存在一种普遍的机制。