Mitochondrial Dysfunction and Biogenesis in Neurodegenerative diseases: Pathogenesis and Treatment.
Mojtaba Golpich,Elham Amini,Zahurin Mohamed,Raymond Azman Ali,Norlinah Mohamed Ibrahim,Abolhassan Ahmadiani +5 more
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TLDR
The purpose of this review was to present the current status of the knowledge and understanding of the involvement of mitochondrial dysfunction in pathogenesis of neurodegenerative diseases including Alzheimer's disease, Parkinson's disease (PD), Huntington’s disease (HD), and amyotrophic lateral sclerosis (ALS) and the importance of mitochondrial biogenesis as a potential novel therapeutic target for their treatment.Abstract:
Neurodegenerative diseases are a heterogeneous group of disorders that are incurable and characterized by the progressive degeneration of the function and structure of the central nervous system (CNS) for reasons that are not yet understood. Neurodegeneration is the umbrella term for the progressive death of nerve cells and loss of brain tissue. Because of their high energy requirements, neurons are especially vulnerable to injury and death from dysfunctional mitochondria. Widespread damage to mitochondria causes cells to die because they can no longer produce enough energy. Several lines of pathological and physiological evidence reveal that impaired mitochondrial function and dynamics play crucial roles in aging and pathogenesis of neurodegenerative diseases. As mitochondria are the major intracellular organelles that regulate both cell survival and death, they are highly considered as a potential target for pharmacological-based therapies. The purpose of this review was to present the current status of our knowledge and understanding of the involvement of mitochondrial dysfunction in pathogenesis of neurodegenerative diseases including Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD), and amyotrophic lateral sclerosis (ALS) and the importance of mitochondrial biogenesis as a potential novel therapeutic target for their treatment. Likewise, we highlight a concise overview of the key roles of mitochondrial electron transport chain (ETC.) complexes as well as mitochondrial biogenesis regulators regarding those diseases.read more
Citations
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Spinal Cord Injury: Pathophysiology, Multimolecular Interactions, and Underlying Recovery Mechanisms.
Anam Anjum,Muhammad Dain Yazid,Muhammad Fauzi Daud,Jalilah Idris,Angela Min Hwei Ng,Amaramalar Selvi Naicker,Ramesh Kumar,Yogeswaran Lokanathan +7 more
TL;DR: Current understanding in this area must be assessed to formulate appropriate treatment modalities to improve SCI recovery, and the understanding of SCI pathophysiology, interrelated or interlinked multimolecular interactions and various methods of neuronal recovery i.e., neuroprotective, immunomodulatory and neuro-regenerative pathways and relevant approaches are promoted.
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Converging pathways in neurodegeneration, from genetics to mechanisms.
TL;DR: The intricate genotype–phenotype relationships and common cellular pathways emerging from recent genetic and mechanistic studies are reviewed, revealing shared pathogenic mechanisms and emerging therapeutic opportunities and challenges.
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The Peroxisome-Mitochondria Connection: How and Why?
TL;DR: A comprehensive look at how peroxisomal and mitochondrial abundance are controlled by common sets of cis- and trans-acting factors and how these organelles cooperate in various metabolic and signaling pathways is provided.
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Mitochondrial biogenesis: An update
TL;DR: A view is provided on the role of mitochondrial biogenesis in homeostasis of the mitochondrial mass and function, the signalling pathways beyond the induction/promotion, stimulation and inhibition of mitochondria, and the therapeutic applications aiming the repair and regeneration of defective mitochondrial biogenic (in ageing, metabolic diseases, neurodegeneration and cancer).
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Shared molecular and cellular mechanisms of premature ageing and ageing-associated diseases
Nard Kubben,Tom Misteli +1 more
TL;DR: Using the rare premature ageing disorder Hutchinson–Gilford progeria syndrome as a paradigm, the shared mechanisms between premature ageing and ageing-associated diseases are discussed, including defects in genetic, epigenetic and metabolic pathways; mitochondrial and protein homeostasis; cell cycle; and stem cell-regenerative capacity.
References
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Sirtuin 3, a New Target of PGC-1α, Plays an Important Role in the Suppression of ROS and Mitochondrial Biogenesis
Xingxing Kong,Rui Wang,Yuan Xue,Xiaojun Liu,Huabing Zhang,Yong Chen,Fude Fang,Yongsheng Chang +7 more
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Mechanisms of Mitochondrial Fission and Fusion
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Journal ArticleDOI
An In Vitro Model of Parkinson's Disease: Linking Mitochondrial Impairment to Altered α-Synuclein Metabolism and Oxidative Damage
Todd B. Sherer,Ranjita Betarbet,Amy K. Stout,Serena Lund,Melisa J. Baptista,Alexander Panov,Mark R. Cookson,J. Timothy Greenamyre +7 more
TL;DR: These studies indicate that chronic low-grade complex I inhibition caused by rotenone exposure induces accumulation and aggregation of α-synuclein and ubiquitin, progressive oxidative damage, and caspase-dependent death, mechanisms that may be central to PD pathogenesis.
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The estrogen-related receptor α (ERRα) functions in PPARγ coactivator 1α (PGC-1α)-induced mitochondrial biogenesis
Sylvia N. Schreiber,Roger Emter,M. Benjamin Hock,Darko Knutti,Darko Knutti,Jessica Cardenas,Michael Podvinec,Edward J. Oakeley,Anastasia Kralli +8 more
TL;DR: This paper showed that ERRα is an effector of the transcriptional coactivator PGC-1α and that it regulates the expression of genes involved in oxidative phosphorylation and mitochondrial biogenesis.
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