Reolon Gustavo Kellermann, Braga Luisa Macedo, Camassola Melissa, Luft Tatiana, Henriques João Antônio Pêgas, Nardi Nance Beyer, Roesler Rafael
Cellular and Molecular Pharmacology Research Group, Department of Pharmacology, Institute for Basic Health Sciences, Federal University of Rio Grande do Sul, 90046-900 Porto Alegre, RS, Brazil.
Brain Res. 2006 Mar 3;1076(1):225-30. doi: 10.1016/j.brainres.2006.01.008. Epub 2006 Feb 13.
Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disease that leads to neurodegeneration and neurological deficits, among other pathological and clinical consequences. The aim of the present study was to evaluate neurobehavioral parameters in a genetic mouse model of mucopolysaccharidosis type I (MPS I). During exploration of an open field, adult MPS I (Idua(-/-)) mice showed normal locomotion and anxiety but reduced number of rearings. Idua(-/-) mice performed normally in a novel object recognition memory task and showed normal short-term retention of inhibitory avoidance training. By contrast, long-term retention of inhibitory avoidance was impaired in Idua(-/-) mice. The deficit in inhibitory avoidance memory could not be attributed to reduced footshock reactivity. The results indicate that Idua(-/-) mice present deficits in long-term memory for aversive training and reduced exploratory behavior.
I型黏多糖贮积症（MPS I）是一种溶酶体贮积病，会导致神经退行性变和神经功能缺损以及其他病理和临床后果。本研究的目的是评估I型黏多糖贮积症（MPS I）基因小鼠模型中的神经行为参数。在旷场探索过程中，成年MPS I（Idua(-/-)）小鼠表现出正常的运动能力和焦虑水平，但直立次数减少。Idua(-/-)小鼠在新物体识别记忆任务中表现正常，并且在抑制性回避训练的短期记忆方面也表现正常。相比之下，Idua(-/-)小鼠在抑制性回避的长期记忆方面受损。抑制性回避记忆的缺陷不能归因于足部电击反应性降低。结果表明，Idua(-/-)小鼠在厌恶训练的长期记忆方面存在缺陷，并且探索行为减少。
