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首页> 外文期刊>Brain research >Hyperactivity, neuromotor defects, and impaired learning and memory in a mouse model for metachromatic leukodystrophy.
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Hyperactivity, neuromotor defects, and impaired learning and memory in a mouse model for metachromatic leukodystrophy.

机译:多动性白细胞营养不良的小鼠模型中的过度活跃,神经运动缺陷和学习与记忆障碍。

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摘要

Deficiency of arylsulfatase A (ASA) causes the autosomal recessive lipidosis, metachromatic leukodystrophy (MLD). Performance on tests of activity, motor ability and learning/memory was assessed in ASA-deficient mice and normal controls at 3, 6 and 12 months-of-age. ASA-deficient mice showed consistently increased cage activity in all age groups, whereas open field activity was increased only in the 3-month-old group. Motor coordination and equilibrium, as tested in the rotarod test, was impaired in 12-month-old ASA-deficient mice. Passive avoidance learning was tested in the step-through box. Performance on this test was impaired in the 12-month-old group only. Spatial learning and memory abilities were tested in the Morris water maze. Six-month-old ASA-deficient mice displayed slightly impaired hidden-platform acquisition performance. Three-month-old animals, on the other hand, did not show any acquisition or retention defect on this task, notwithstanding significantly reduced swimming velocity. Acquisition training, both in the hidden- and visible-platform conditions of the Morris water maze, and retention performance during the probe trials were impaired in 12-month-old ASA-deficient mice. The hyperactivity, motor incoordination and slowing, and the age-related learning/memory defects, reported here in ASA-deficient mice, may relate to the decline of neuromotor and cognitive functions in MLD patients, and could be used as correlative or outcome measures in the study of MLD pathophysiology and treatment.
机译:芳基硫酸酯酶A(ASA)的缺乏会导致常染色体隐性脂质增生,异色性白细胞营养不良(MLD)。在3、6和12个月大的ASA缺陷小鼠和正常对照组中评估了对活动,运动能力和学习/记忆的测试表现。缺乏ASA的小鼠在所有年龄组中均显示出笼养活动持续增加,而仅在3个月大组中,开放视野活动有所增加。在12个月大的ASA缺陷小鼠中,如在轮转试验中测试的那样,运动协调和平衡受到损害。在逐步练习中测试了被动回避学习。仅在12个月大的组中,该测试的性能受到了损害。在莫里斯水迷宫中测试了空间学习和记忆能力。六个月大的ASA缺陷小鼠显示出隐藏平台的采集性能略有受损。另一方面,尽管游泳速度明显降低,但三个月大的动物在该任务上没有表现出任何获取或保留缺陷。在12个月大的ASA缺陷小鼠中,在莫里斯水迷宫的隐藏平台和可见平台条件下进行的获取训练和探针试验期间的保持性能均受到损害。在ASA缺陷小鼠中报道的多动,运动不协调和减慢以及与年龄相关的学习/记忆缺陷可能与MLD患者的神经运动和认知功能下降有关,并且可以用作相关或结局指标MLD病理生理和治疗的研究。

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