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环境富集改善雷特综合征小鼠模型中的运动协调缺陷——Mecp2基因剂量效应和脑源性神经营养因子表达

Environmental enrichment ameliorates a motor coordination deficit in a mouse model of Rett syndrome--Mecp2 gene dosage effects and BDNF expression.

作者信息

Kondo Mari, Gray Laura J, Pelka Gregory J, Christodoulou John, Tam Patrick P L, Hannan Anthony J

机构信息

Howard Florey Institute and Department of Anatomy and Cell Biology, University of Melbourne, Melbourne, VIC, Australia.

出版信息

Eur J Neurosci. 2008 Jun;27(12):3342-50. doi: 10.1111/j.1460-9568.2008.06305.x. Epub 2008 Jun 14.

Abstract

Rett syndrome, commonly associated with mutations of the methyl CpG-binding protein 2 (MECP2) gene, is characterised by an apparently normal early postnatal development followed by deterioration of acquired cognitive and motor coordination skills in early childhood. To evaluate whether environmental factors may influence the disease outcome of Rett syndrome, we tested the effect of environmental enrichment from 4 weeks of age on the behavioural competence of mutant mice harboring a Mecp2 (tm1Tam)-null allele. Our findings show that enrichment improves motor coordination in heterozygous Mecp2+/- females but not Mecp2-/y males. Standard-housed Mecp2+/- mice had an initial motor coordination deficit on the accelerating rotarod, which improved with training then deteriorated in subsequent weeks. Enrichment resulted in a significant reduction in this coordination deficit in Mecp2+/- mice, returning the performance to wild-type levels. Brain-derived neurotrophic factor (BDNF) protein levels were 75 and 85% of wild-type controls in standard-housed and environmentally enriched Mecp2+/- cerebellum, respectively. Mecp2-/y mice showed identical deficits of cerebellar BDNF (67% of wild-type controls) irrespective of their housing environment. Our findings demonstrate a positive impact of environmental enrichment in a Rett syndrome model; this impact may be dependent on the existence of one functional copy of Mecp2.

摘要

瑞特综合征通常与甲基CpG结合蛋白2(MECP2)基因突变有关,其特征是出生后早期发育明显正常,随后在幼儿期后天获得的认知和运动协调技能逐渐退化。为了评估环境因素是否可能影响瑞特综合征的疾病转归,我们测试了从4周龄开始进行环境丰富化处理对携带Mecp2(tm1Tam)无效等位基因的突变小鼠行为能力的影响。我们的研究结果表明,环境丰富化改善了杂合子Mecp2+/-雌性小鼠的运动协调能力,但对Mecp2-/y雄性小鼠没有影响。饲养在标准环境中的Mecp2+/-小鼠在加速旋转棒试验中最初存在运动协调缺陷,训练后有所改善,但在随后几周又恶化。环境丰富化导致Mecp2+/-小鼠的这种协调缺陷显著减少,使其表现恢复到野生型水平。在饲养于标准环境和环境丰富化环境中的Mecp2+/-小鼠的小脑中,脑源性神经营养因子(BDNF)蛋白水平分别为野生型对照的75%和85%。无论饲养环境如何,Mecp2-/y小鼠的小脑BDNF均表现出相同的缺陷(为野生型对照的67%)。我们的研究结果证明了环境丰富化对瑞特综合征模型有积极影响;这种影响可能取决于Mecp2一个功能拷贝的存在。

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