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首页> 外文期刊>Biochemical Genetics >Biochemical and Behavioral Evaluation of Human MAPT Mutations in Transgenic Drosophila melanogaster
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Biochemical and Behavioral Evaluation of Human MAPT Mutations in Transgenic Drosophila melanogaster

机译：人类MAPT突变在果蝇中的生化和行为评价。

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摘要

Mutations in the human microtubule-associated protein tau (hMAPT) gene including R406W and V337M result in autosomal dominant neurodegenerative disorder. These mutations lead to hyperphosphorylation and aggregation of Tau protein which is a known genetic factor underlying development of Alzheimer's disease (AD). In the present study, transgenic Drosophila models of AD expressing wild-type and mutant forms of hMAPT exhibit a progressive neurodegeneration which was manifested in the form of early death and impairment of cognitive ability. Moreover, they were also found to have significantly decreased activity of neurotransmitter enzymes accompanied by decreased cellular endogenous antioxidant profile. The extent of neurodegeneration, memory impairment, and biochemical profiles was different in the tau transgenic strains which indicate multiple molecular and cellular responses underlie each particular form of hMAPT.
机译：人类微管相关蛋白tau（hMAPT）基因中的突变（包括R406W和V337M）导致常染色体显性遗传性神经退行性疾病。这些突变导致Tau蛋白的过度磷酸化和聚集，Tau蛋白是阿尔茨海默氏病（AD）发展的已知遗传因素。在本研究中，表达hMAPT的野生型和突变形式的AD转基因果蝇模型表现出进行性神经变性，表现为早期死亡和认知能力受损。此外，还发现它们的神经递质酶活性显着降低，同时细胞内源性抗氧化剂谱降低。在tau转基因菌株中，神经变性，记忆障碍和生化特征的程度不同，这表明hMAPT的每种特定形式均基于多种分子和细胞反应。

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