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
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Antibodies to the RNA binding protein heterogeneous nuclear ribonucleoprotein A1 contribute to neuronal cell loss in an animal model of multiple sclerosis
TL;DR: It is shown that injection of anti‐hnRNP A1 antibodies, in contrast to control antibodies, resulted in worsened disease and increased neurodegeneration in experimental autoimmune encephalomyelitis (EAE), suggesting that autoimmunity to RBPs, such as hnR NP A1, play a role in neurodegenersation in EAE with important implications for the pathogenesis of MS.
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Effects of endurance training on skeletal muscle mitochondrial function in Huntington disease patients
Sandro Manuel Mueller,Saskia Maria Gehrig,Jens A. Petersen,Sebastian Frese,Violeta Mihaylova,Maria Ligon-Auer,Natalia Khmara,Jean-Marc Nuoffer,André Schaller,Carsten Lundby,Marco Toigo,Marco Toigo,Hans H. Jung +12 more
TL;DR: Endurance training is a safe and feasible option to enhance indices of energy metabolism in skeletal muscle of HD patients and may represent a potential therapeutic approach to delay the onset and/or progression of muscular dysfunction.
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Identification of dual Sigma1 receptor modulators/acetylcholinesterase inhibitors with antioxidant and neurotrophic properties, as neuroprotective agents.
Marta Rui,Giacomo Rossino,Stefania Coniglio,Stefania Monteleone,Arianna Scuteri,Alessio Malacrida,Daniela Rossi,Laura Catenacci,Milena Sorrenti,Mayra Paolillo,Daniela Curti,Letizia Venturini,Dirk Schepmann,Bernhard Wünsch,Klaus R. Liedl,Guido Cavaletti,Vittorio Pace,Ernst Urban,Simona Collina +18 more
TL;DR: The design, synthesis and evaluation of dual Sigma 1 Receptor (S1R) modulators/Acetylcholinesterase (AChE) inhibitors endowed with antioxidant and neurotrophic properties, potentially able to counteract neurodegeneration are reported on.
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Therapeutic Potential of Hispidin-Fungal and Plant Polyketide.
Kseniia A. Palkina,Daria A. Ipatova,Daria A. Ipatova,Ekaterina S. Shakhova,Anastasia V. Balakireva,Nadezhda M. Markina +5 more
TL;DR: A review of 20 years of hispidin studies of its antioxidant, anti-inflammatory, antiapoptotic, antiviral, and anti-cancer cell activity is presented in this paper.
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Amyotrophic lateral sclerosis alters the metabolic aging profile in patient derived fibroblasts.
Margarita Gerou,Benjamin A. Hall,Ryan Woof,Jessica Allsop,Stephen J. Kolb,Kathrin Meyer,Pamela J. Shaw,Scott P. Allen +7 more
TL;DR: In this article, the effect of aging on the metabolic profile of fibroblasts derived from ALS cases compared to controls was examined using a newly established phenotypic metabolic approach, and the results suggest that supplementing those pathways may protect against age related metabolic dysfunction in ALS.
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