Mitochondria Dysfunction in Frontotemporal Dementia/Amyotrophic Lateral Sclerosis: Lessons From Drosophila Models.
Mitochondria Dysfunction in Frontotemporal Dementia/Amyotrophic Lateral Sclerosis: Lessons From Drosophila Models.
复制标题
额颞叶痴呆/肌萎缩侧索硬化症的线粒体功能障碍：来自果蝇模型的教训。
DOI：
10.3389/fnins.2021.786076
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发表时间：
2021
影响因子：
4.3
通讯作者：
Niccoli T
中科院分区：
文献类型：
--
作者：
Anoar S;Woodling NS;Niccoli T
额颞叶痴呆（FTD）和肌萎缩侧索硬化症（ALS）是神经退行性疾病，其特征是运动和认知功能下降。尽管这些疾病表现出不同的症状，但 FTD 和 ALS 是同一疾病谱的两个极端，因为它们在遗传、临床和神经病理学特征上表现出相当大的重叠。在这些重叠的特征中，线粒体功能障碍与 FTD 和 ALS 相关。最近的研究表明，源自患者诱导多能干细胞（iPSC）的细胞表现出线粒体异常，并且在许多动物疾病模型中也观察到了类似的异常。果蝇模型因其快速的生成时间和广泛的遗传工具而被广泛用于研究 FTD 和 ALS。人们已经开发了多种果蝇模型来阐明与 FTD/ALS 相关的突变毒性的分子机制。果蝇模型通常有助于理解线粒体生物学中与疾病相关的突变的作用。在这篇综述中，我们讨论了与 FTD/ALS 相关的突变如何破坏线粒体功能，并回顾了果蝇模型的使用如何对我们当前在该领域的知识至关重要。
Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are neurodegenerative disorders characterized by declining motor and cognitive functions. Even though these diseases present with distinct sets of symptoms, FTD and ALS are two extremes of the same disease spectrum, as they show considerable overlap in genetic, clinical and neuropathological features. Among these overlapping features, mitochondrial dysfunction is associated with both FTD and ALS. Recent studies have shown that cells derived from patients’ induced pluripotent stem cells (iPSC)s display mitochondrial abnormalities, and similar abnormalities have been observed in a number of animal disease models. Drosophila models have been widely used to study FTD and ALS because of their rapid generation time and extensive set of genetic tools. A wide array of fly models have been developed to elucidate the molecular mechanisms of toxicity for mutations associated with FTD/ALS. Fly models have been often instrumental in understanding the role of disease associated mutations in mitochondria biology. In this review, we discuss how mutations associated with FTD/ALS disrupt mitochondrial function, and we review how the use of Drosophila models has been pivotal to our current knowledge in this field.
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影响因子：
16.2
作者：
Ash PE;Bieniek KF;Gendron TF;Caulfield T;Lin WL;Dejesus-Hernandez M;van Blitterswijk MM;Jansen-West K;Paul JW 3rd;Rademakers R;Boylan KB;Dickson DW;Petrucelli L
通讯作者：
Petrucelli L
影响因子：
2.4
作者：
Agudelo A;St Amand V;Grissom L;Lafond D;Achilli T;Sahin A;Reenan R;Stilwell G
通讯作者：
Stilwell G
影响因子：
3.5
作者：
Baldwin KR;Godena VK;Hewitt VL;Whitworth AJ
通讯作者：
Whitworth AJ
影响因子：
13.6
作者：
Bangi, Erdem;Ang, Celina;Cagan, Ross L.
通讯作者：
Cagan, Ross L.
影响因子：
4
作者：
Ayala, Youhna M.;Zago, Paola;Baralle, Francisco E.
通讯作者：
Baralle, Francisco E.