Graduate Interdisciplinary Program in Neuroscience, University of Arizona, Tucson, Arizona, United State of America.
Department of Neuroscience, University of Arizona, Tucson, Arizona, United States of America.
PLoS One. 2022 May 4;17(5):e0265604. doi: 10.1371/journal.pone.0265604. eCollection 2022.
Deterioration in the quality of a person's voice and speech is an early marker of Parkinson's disease (PD). In humans, the neural circuit that supports vocal motor control consists of a cortico-basal ganglia-thalamo-cortico loop. The basal ganglia regions, striatum and globus pallidus, in this loop play a role in modulating the acoustic features of vocal behavior such as loudness, pitch, and articulatory rate. In PD, this area is implicated in pathogenesis. In animal models of PD, the accumulation of toxic aggregates containing the neuronal protein alpha-synuclein (αsyn) in the midbrain and striatum result in limb and vocal motor impairments. It has been challenging to study vocal impairments given the lack of well-defined cortico-basal ganglia circuitry for vocalization in rodent models. Furthermore, whether deterioration of voice quality early in PD is a direct result of αsyn-induced neuropathology is not yet known. Here, we take advantage of the well-characterized vocal circuits of the adult male zebra finch songbird to experimentally target a song-dedicated pathway, the anterior forebrain pathway, using an adeno-associated virus expressing the human wild-type αsyn gene, SNCA. We found that overexpression of αsyn in this pathway coincides with higher levels of insoluble, monomeric αsyn compared to control finches. Impairments in song production were also detected along with shorter and poorer quality syllables, which are the most basic unit of song. These vocal changes are similar to the vocal abnormalities observed in individuals with PD.
人的声音和言语质量的恶化是帕金森病(PD)的早期标志。在人类中,支持发声运动控制的神经回路由皮质基底节-丘脑-皮质回路组成。该回路中的基底节区域,纹状体和苍白球,在调节发声行为的声学特征(如响度、音高和发音速度)方面发挥作用。在 PD 中,该区域与发病机制有关。在 PD 的动物模型中,含有神经元蛋白α-突触核蛋白(αsyn)的毒性聚集体在中脑和纹状体中的积累导致肢体和发声运动障碍。由于啮齿动物模型中缺乏明确的皮质基底节发声回路,因此研究发声障碍具有挑战性。此外,PD 早期声音质量的恶化是否是αsyn 诱导的神经病理学的直接结果尚不清楚。在这里,我们利用成年雄性斑马雀鸣禽的特征明确的发声回路,通过表达人类野生型αsyn 基因 SNCA 的腺相关病毒靶向专门用于歌唱的通路,即前脑通路。我们发现,与对照雀鸟相比,该通路中αsyn 的过表达与更高水平的不溶性单体αsyn 同时出现。还检测到歌唱产生的障碍以及更短和质量更差的音节,这是歌唱的最基本单元。这些发声变化与 PD 个体中观察到的发声异常相似。