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靶向脑特异性tau蛋白病会损害外周骨骼肌的完整性和功能。

Targeted brain-specific tauopathy compromises peripheral skeletal muscle integrity and function.

作者信息

Alava Bryan, Hery Gabriela, Sidhom Silvana, Prokop Stefan, Esser Karyn, Abisambra Jose

机构信息

Department of Physiology and Aging, University of Florida, Gainesville, Florida, 32610, USA.

Center for Translational Research in Neurodegenerative Disease (CTRND), University of Florida, Gainesville, Florida, 32610, USA.

出版信息

bioRxiv. 2023 Nov 17:2023.11.17.567586. doi: 10.1101/2023.11.17.567586.

Abstract

Tauopathies are neurodegenerative disorders in which the pathological intracellular aggregation of the protein tau causes cognitive deficits. Additionally, clinical studies report muscle weakness in populations with tauopathy. However, whether neuronal pathological tau species confer muscle weakness, and whether skeletal muscle maintains contractile capacity in primary tauopathy remains unknown. Here, we identified skeletal muscle abnormalities in a mouse model of primary tauopathy, expressing human mutant P301L-tau using adeno-associated virus serotype 8 (AAV8). AAV8-P301L mice showed grip strength deficits, hyperactivity, and abnormal histological features of skeletal muscle. Additionally, spatially resolved gene expression of muscle cross sections were altered in AAV8-P301L myofibers. Transcriptional changes showed alterations of genes encoding sarcomeric proteins, proposing a weakness phenotype. Strikingly, specific force of the soleus muscle was blunted in AAV8-P301L tau male mice. Our findings suggest tauopathy has peripheral consequences in skeletal muscle that contribute to weakness in tauopathy.

摘要

tau蛋白病是一类神经退行性疾病,其中tau蛋白在细胞内的病理性聚集会导致认知缺陷。此外,临床研究报告称tau蛋白病患者存在肌肉无力的症状。然而,神经元病理性tau蛋白种类是否会导致肌肉无力,以及在原发性tau蛋白病中骨骼肌是否保持收缩能力,目前仍不清楚。在此,我们在原发性tau蛋白病小鼠模型中发现了骨骼肌异常,该模型通过腺相关病毒血清型8(AAV8)表达人类突变型P301L-tau蛋白。AAV8-P301L小鼠表现出握力缺陷、多动以及骨骼肌的组织学特征异常。此外,AAV8-P301L肌纤维中肌肉横截面的空间分辨基因表达发生了改变。转录变化显示编码肌节蛋白的基因发生了改变,提示存在虚弱表型。引人注目的是,AAV8-P301L tau雄性小鼠比目鱼肌的比肌力减弱。我们的研究结果表明,tau蛋白病对骨骼肌有外周影响,这是导致tau蛋白病患者肌肉无力的原因之一。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b125/10680826/191b130317fc/nihpp-2023.11.17.567586v1-f0001.jpg

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