Clemensson Erik Karl Håkan, Novati Arianna, Clemensson Laura Emily, Riess Olaf, Nguyen Huu Phuc
University of Tuebingen, Institute of Medical Genetics and Applied Genomics, Calwerstrasse 7, 72076 Tuebingen, Germany; University of Tuebingen, Centre for Rare Diseases, Calwerstrasse 7, 72076 Tuebingen, Germany.
University of Tuebingen, Institute of Medical Genetics and Applied Genomics, Calwerstrasse 7, 72076 Tuebingen, Germany; University of Tuebingen, Centre for Rare Diseases, Calwerstrasse 7, 72076 Tuebingen, Germany.
Behav Brain Res. 2019 Feb 1;359:116-126. doi: 10.1016/j.bbr.2018.10.036. Epub 2018 Oct 29.
Huntington disease is a hereditary neurodegenerative disease, in which patients display a broad range of clinical symptoms. Among these, impaired inhibitory control has been noted. The BACHD rat is a recently developed and established transgenic animal model for Huntington disease, and characterizing the presence of Huntington disease-like behavioural phenotypes in these animals is of importance. Prior studies have indicated that BACHD rats suffer from impaired inhibitory control, although further studies are necessary to fully understand the scope and specific nature of these phenotypes. In the current study, BACHD rats were trained to perform a Go/No-Go-like test of visual discrimination, akin to behavioural tests that have revealed suspected response inhibition impairments in Huntington disease patients. The results indicate that although BACHD rats showed a slow rate of learning to inhibit responses on No-Go trials, once they had learned to handle the basic discrimination, they had an unchanged ability to withhold lever responses during extended periods of time. This suggests that BACHD rats have specific impairments when applying inhibitory control to a new or changed situation. The findings are in line with previous studies of BACHD rats and support the continued use and characterization of this animal model.
亨廷顿病是一种遗传性神经退行性疾病，患者会表现出广泛的临床症状。其中，抑制控制受损已被注意到。BACHD大鼠是最近开发并建立的亨廷顿病转基因动物模型，在这些动物中表征亨廷顿病样行为表型的存在具有重要意义。先前的研究表明，BACHD大鼠存在抑制控制受损的情况，尽管需要进一步研究以全面了解这些表型的范围和具体性质。在当前研究中，训练BACHD大鼠进行视觉辨别类似的“去/不去”测试，类似于已揭示亨廷顿病患者疑似反应抑制受损的行为测试。结果表明，尽管BACHD大鼠在“不去”试验中学习抑制反应的速度较慢，但一旦它们学会处理基本辨别，它们在较长时间内抑制杠杆反应的能力不变。这表明BACHD大鼠在将抑制控制应用于新的或变化的情况时存在特定损伤。这些发现与先前对BACHD大鼠的研究一致，并支持继续使用和表征这种动物模型。
