Mitochondria Dysfunction in Frontotemporal Dementia/Amyotrophic Lateral Sclerosis: Lessons From Drosophila Models

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 c...

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Autores principales:	Sharifah Anoar, Nathaniel S. Woodling, Teresa Niccoli
Formato:	article
Lenguaje:	EN
Publicado:	Frontiers Media S.A. 2021
Materias:	Drosophila neurodegeneration mitochondria ALS FTD Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571
Acceso en línea:	https://doaj.org/article/e24708bfb6ef4f1f8fe028afc984ac79
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spelling	oai:doaj.org-article:e24708bfb6ef4f1f8fe028afc984ac792021-11-30T18:29:34ZMitochondria Dysfunction in Frontotemporal Dementia/Amyotrophic Lateral Sclerosis: Lessons From Drosophila Models1662-453X10.3389/fnins.2021.786076https://doaj.org/article/e24708bfb6ef4f1f8fe028afc984ac792021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fnins.2021.786076/fullhttps://doaj.org/toc/1662-453XFrontotemporal 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.Sharifah AnoarNathaniel S. WoodlingTeresa NiccoliFrontiers Media S.A.articleDrosophilaneurodegenerationmitochondriaALSFTDNeurosciences. Biological psychiatry. NeuropsychiatryRC321-571ENFrontiers in Neuroscience, Vol 15 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Drosophila neurodegeneration mitochondria ALS FTD Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571
spellingShingle	Drosophila neurodegeneration mitochondria ALS FTD Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571 Sharifah Anoar Nathaniel S. Woodling Teresa Niccoli Mitochondria Dysfunction in Frontotemporal Dementia/Amyotrophic Lateral Sclerosis: Lessons From Drosophila Models
description	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.
format	article
author	Sharifah Anoar Nathaniel S. Woodling Teresa Niccoli
author_facet	Sharifah Anoar Nathaniel S. Woodling Teresa Niccoli
author_sort	Sharifah Anoar
title	Mitochondria Dysfunction in Frontotemporal Dementia/Amyotrophic Lateral Sclerosis: Lessons From Drosophila Models
title_short	Mitochondria Dysfunction in Frontotemporal Dementia/Amyotrophic Lateral Sclerosis: Lessons From Drosophila Models
title_full	Mitochondria Dysfunction in Frontotemporal Dementia/Amyotrophic Lateral Sclerosis: Lessons From Drosophila Models
title_fullStr	Mitochondria Dysfunction in Frontotemporal Dementia/Amyotrophic Lateral Sclerosis: Lessons From Drosophila Models
title_full_unstemmed	Mitochondria Dysfunction in Frontotemporal Dementia/Amyotrophic Lateral Sclerosis: Lessons From Drosophila Models
title_sort	mitochondria dysfunction in frontotemporal dementia/amyotrophic lateral sclerosis: lessons from drosophila models
publisher	Frontiers Media S.A.
publishDate	2021
url	https://doaj.org/article/e24708bfb6ef4f1f8fe028afc984ac79
work_keys_str_mv	AT sharifahanoar mitochondriadysfunctioninfrontotemporaldementiaamyotrophiclateralsclerosislessonsfromdrosophilamodels AT nathanielswoodling mitochondriadysfunctioninfrontotemporaldementiaamyotrophiclateralsclerosislessonsfromdrosophilamodels AT teresaniccoli mitochondriadysfunctioninfrontotemporaldementiaamyotrophiclateralsclerosislessonsfromdrosophilamodels
_version_	1718406416696344576

Mitochondria Dysfunction in Frontotemporal Dementia/Amyotrophic Lateral Sclerosis: Lessons From Drosophila Models

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