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Mitochondria, parkinson's disease

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https://www.readbyqxmd.com/read/29626647/multiple-pathways-for-mitophagy-a-neurodegenerative-conundrum-for-parkinson-s-disease
#1
REVIEW
Charleen T Chu
It has been nearly a decade since the first landmark studies implicating familial recessive Parkinson's disease genes in the regulation of selective mitochondrial autophagy. The PTEN-induced kinase 1 (PINK1) and the E3 ubiquitin ligase Parkin (encoded by the PARK2 gene) act together to mark depolarized mitochondria for degradation. There is now an extensive body of literature detailing key mediators and steps in this pathway, based mostly on work in transformed cell lines. However, the degree to which PINK1-triggered mitophagy contributes to mitochondrial quality control in the mammalian brain, and the extent to which its disruption contributes to Parkinson's disease pathogenesis remain uncertain...
April 4, 2018: Neuroscience Letters
https://www.readbyqxmd.com/read/29624777/de-novo-tetrahydrobiopterin-biosynthesis-is-impaired-in-the-inflammed-striatum-of-parkin-mice
#2
Roberta de Paula Martins, Viviane Glaser, Aderbal S Aguiar, Priscila Maximiliano de Paula Ferreira, Karina Ghisoni, Débora da Luz Scheffer, Laurence Lanfumey, Rita Raisman-Vozari, Olga Corti, Ana Lucia De Paul, Rodrigo Augusto da Silva, Alexandra Latini
Parkinson's disease (PD), the second-most prevalent neurodegenerative disease, is primarily characterized by neurodegeneration in the substantia nigra pars compacta, resulting in motor impairment. Loss-of-function mutations in parkin are the major cause of the early-onset familial form of the disease. Although rodents deficient in parkin (parkin(-/-)) have some dopaminergic system dysfunction associated with central oxidative stress and energy metabolism deficiencies, these animals only display nigrostriatal pathway degeneration under inflammatory conditions...
April 6, 2018: Cell Biology International
https://www.readbyqxmd.com/read/29619740/nicotine-modulates-mitochondrial-dynamics-in-hippocampal-neurons
#3
Juan A Godoy, Angel G Valdivieso, Nibaldo C Inestrosa
Mitochondria are widely recognized as fundamental organelles for cellular physiology and constitute the main energy source for different cellular processes. The location, morphology, and interactions of mitochondria with other organelles, such as the endoplasmic reticulum (ER), have emerged as critical events capable of determining cellular fate. Mitochondria-related functions have proven particularly relevant in neurons; mitochondria are necessary for proper neuronal morphogenesis and the highly energy-demanding synaptic transmission process...
April 4, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29616350/mitochondrial-dysfunction-in-parkinson-s-disease-new-mechanistic-insights-and-therapeutic-perspectives
#4
REVIEW
Jin-Sung Park, Ryan L Davis, Carolyn M Sue
PURPOSE OF REVIEW: Parkinson's disease (PD) is a complex neurodegenerative disorder, the aetiology of which is still largely unknown. Overwhelming evidence indicates that mitochondrial dysfunction is a central factor in PD pathophysiology. Here we review recent developments around mitochondrial dysfunction in familial and sporadic PD, with a brief overview of emerging therapies targeting mitochondrial dysfunction. RECENT FINDINGS: Increasing evidence supports the critical role for mitochondrial dysfunction in the development of sporadic PD, while the involvement of familial PD-related genes in the regulation of mitochondrial biology has been expanded by the discovery of new mitochondria-associated disease loci and the identification of their novel functions...
April 3, 2018: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/29604226/stem-cell-modeling-of-mitochondrial-parkinsonism-reveals-key-functions-of-opa1
#5
Mindaugas Jonikas, Martin Madill, Alexandre Mathy, Theresa Zekoll, Christos E Zois, Simon Wigfield, Marzena Kurzawa-Akanbi, Cathy Browne, David Sims, Patrick F Chinnery, Sally A Cowley, George K Tofaris
OBJECTIVE: Defective mitochondrial function due to OPA1 mutations causes primarily optic atrophy and less commonly neurodegenerative syndromes. The pathomechanism by which OPA1 mutations trigger diffuse loss of neurons in some but not all patients is unknown. Here we used a tractable iPSC-based model to capture the biology of OPA1 haploinsufficiency in cases presenting with classic eye disease versus syndromic parkinsonism. METHODS: iPSC were generated from two patients with OPA1 haploinsufficiency and two controls and differentiated into dopaminergic neurons...
March 31, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29599708/distinct-mechanisms-of-pathogenic-dj-1-mutations-in-mitochondrial-quality-control
#6
Daniela Strobbe, Alexis A Robinson, Kirsten Harvey, Lara Rossi, Caterina Ferraina, Valerio de Biase, Carlo Rodolfo, Robert J Harvey, Michelangelo Campanella
The deglycase and chaperone protein DJ-1 is pivotal for cellular oxidative stress responses and mitochondrial quality control. Mutations in PARK7 , encoding DJ-1, are associated with early-onset familial Parkinson's disease and lead to pathological oxidative stress and/or disrupted protein degradation by the proteasome. The aim of this study was to gain insights into the pathogenic mechanisms of selected DJ-1 missense mutations, by characterizing protein-protein interactions, core parameters of mitochondrial function, quality control regulation via autophagy, and cellular death following dopamine accumulation...
2018: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29588162/aminochrome-decreases-ngf-gdnf-and-induces-neuroinflammation-in-organotypic-midbrain-slice-cultures
#7
Fillipe M de Araújo, Rafael S Ferreira, Cleide S Souza, Cleonice Creusa Dos Santos, Tácio L R S Rodrigues, Juliana Helena C E Silva, Juciano Gasparotto, Daniel Pens Gelain, Ramon S El-Bachá, Maria de Fátima D Costa, José Claudio M Fonseca, Juan Segura-Aguilar, Silvia L Costa, Victor Diogenes A Silva
Recent evidence shows that aminochrome induces glial activation related to neuroinflammation. This dopamine derived molecule induces formation and stabilization of alpha-synuclein oligomers, mitochondria dysfunction, oxidative stress, dysfunction of proteasomal and lysosomal systems, endoplasmic reticulum stress and disruption of the microtubule network, but until now there has been no evidence of effects on production of cytokines and neurotrophic factors, that are mechanisms involved in neuronal loss in Parkinson's disease (PD)...
March 24, 2018: Neurotoxicology
https://www.readbyqxmd.com/read/29581821/unraveling-the-burden-of-iron-in-neurodegeneration-intersections-with-amyloid-beta-peptide-pathology
#8
REVIEW
Romina María Uranga, Gabriela Alejandra Salvador
Iron overload is a hallmark of many neurodegenerative processes such as Alzheimer's, Parkinson's, and Huntington's diseases. Unbound iron accumulated as a consequence of brain aging is highly reactive with water and oxygen and produces reactive oxygen species (ROS) or free radicals. ROS are toxic compounds able to damage cell membranes, DNA, and mitochondria. Which are the mechanisms involved in neuronal iron homeostasis and in neuronal response to iron-induced oxidative stress constitutes a cutting-edge topic in metalloneurobiology...
2018: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/29580919/sirtuins-in-mitochondrial-stress-indispensable-helpers-behind-the-scenes
#9
REVIEW
Shuangzhe Lin, Haiping Xing, Tongtong Zang, Xin Ruan, Lulu Wo, Ming He
Mitochondria play an essential part in guaranteeing normal cellular physiological functions through providing ATP and participating in diverse processes and signaling pathways. Recently, more and more studies have revealed the vital roles of mitochondria in coping with stressors in the aging process, metabolic disturbances and neurological disorders. Mitochondrial stress responses, including the mitochondrial unfolded protein response (UPRmt ), antioxidant defense, mitochondrial fission, mitochondrial fusion and mitophagy, are induced to maintain cellular integrity in response to stress...
March 23, 2018: Ageing Research Reviews
https://www.readbyqxmd.com/read/29570846/neuroprotective-effects-of-creatine-in-the-cmvmjd135-mouse-model-of-spinocerebellar-ataxia-type-3
#10
Sara Duarte-Silva, Andreia Neves-Carvalho, Carina Soares-Cunha, Joana M Silva, Andreia Teixeira-Castro, Rita Vieira, Anabela Silva-Fernandes, Patrícia Maciel
BACKGROUND AND OBJECTIVE: Mitochondrial dysfunction has been implicated in several neurodegenerative diseases. Creatine administration increases concentration of the energy buffer phosphocreatine, exerting protective effects in the brain. We evaluate whether a creatine-enriched diet would be beneficial for a mouse model of spinocerebellar ataxia type 3, a genetically defined neurodegenerative disease for which no treatment is available. METHODS: We performed 2 independent preclinical trials using the CMVMJD135 mouse model (treating 2 groups of animals with different disease severity) and wild-type mice, to which 2% creatine was provided for 19 (preclinical trial 1) or 29 (preclinical trial 2) weeks, starting at a presymptomatic age...
March 23, 2018: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/29563254/phosphorylation-of-mcad-selectively-rescues-pink1-deficiencies-in-behavior-and-metabolism
#11
Meredith M Course, Anna I Scott, Carmen Schoor, Chung-Han Hsieh, Amanda M Papakyrikos, Dominic Winter, Tina M Cowan, Xinnan Wang
PINK1 is a mitochondria-targeted kinase, whose mutations are a cause of Parkinson's disease. We set out to better understand PINK1's effects on mitochondrial proteins in vivo Using an unbiased phosphoproteomic screen in Drosophila , we found that PINK1 mediates the phosphorylation of MCAD, a mitochondrial matrix protein critical to fatty acid metabolism. By mimicking phosphorylation of this protein in a PINK1 null background, we restored PINK1 null's climbing, flight, thorax, and wing deficiencies. Due to MCAD's role in fatty acid metabolism, we examined the metabolic profile of PINK1 null flies, where we uncovered significant disruptions in both acylcarnitines and amino acids...
March 21, 2018: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/29561660/loss-of-parkin-impairs-mitochondrial-function-and-leads-to-muscle-atrophy
#12
Nesibe Peker, Vinay Donipadi, Mridula Sharma, Craig McFarlane, Ravi Kambadur
Parkinson's Disease is a neurodegenerative disease characterized by tremors, muscle stiffness and muscle weakness. Molecular genetic analysis confirmed that mutations in PARKIN and PINK1 genes, which play major roles in mitochondrial quality control and mitophagy, are frequently associated with Parkinson's Disease. PARKIN is an E3 ubiquitin ligase that translocates to mitochondria during loss of mitochondrial membrane potential to increase mitophagy. Although muscle dysfunction is noted in Parkinson's Disease, little is known about the involvement of PARKIN in the muscle phenotype of Parkinson's Disease...
March 21, 2018: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/29546836/single-chain-fv-antibodies-for-targeting-neurodegenerative-diseases
#13
Chye Soi Moi, Chia Kin Yen, Khuen Yen Ng, Koh Rhun Yian
Protein misfolding and aggregation have been considered the common pathological hallmarks for a number of neurodegenerative diseases, including Alzheimer's disease (AD), Parkinson's disease (PD) and Huntington's disease (HD). These abnormal proteins aggregation damage mitochondria and induce oxidative stress and resulting neuronal cell death. Prolong neuronal damage activates microglia and astrocytes, development of inflammation reaction and further promotes neurodegeneration. Thus, elimination of abnormal proteins aggregation without eliciting any adverse effects are the main treatment strategies...
March 15, 2018: CNS & Neurological Disorders Drug Targets
https://www.readbyqxmd.com/read/29538099/mitochondrial-calcium-uniporter-mediated-inhibition-of-1-methyl-4-phenylpyridinium-ions-neurotoxicity-in-pc12-cells
#14
Huiling Wang, Menglan Zhao, Jialong Chen, Yixian Ren, Guanghai Wang, Wenjun Li, Fei Zou
Parkinson's disease (PD) is one of the most debilitating neurodegenerative disorders. The etiology of sporadic PD remains unknown. One prominent hypothesis is that impaired mitochondrial function may underlie slow and progressive neurodegeneration. Mitochondrial calcium uniporter (MCU) is a crucial component that regulates the intramitochondrial Ca level. Ca uptake to the mitochondria by MCU, resulting in activation of mitochondrial dehydrogenases and stimulation of ATP synthesis, but excessive Ca uptake to the mitochondria resulting in cell apoptosis...
March 13, 2018: Neuroreport
https://www.readbyqxmd.com/read/29529199/superoxide-dismutating-molecules-rescue-the-toxic-effects-of-pink1-and-parkin-loss
#15
Alice Biosa, Alvaro Sanchez-Martinez, Roberta Filograna, Ana Terriente-Felix, Sarah M Alam, Mariano Beltramini, Luigi Bubacco, Marco Bisaglia, Alexander J Whitworth
Reactive oxygen species exert important functions in regulating several cellular signalling pathways. However, an excessive accumulation of reactive oxygen species can perturb the redox homeostasis leading to oxidative stress, a condition which has been associated to many neurodegenerative disorders. Accordingly, alterations in the redox state of cells and mitochondrial homeostasis are established hallmarks in both familial and sporadic Parkinson's disease cases. PINK1 and parkin are two genes which account for a large fraction of autosomal recessive early-onset forms of Parkinson's disease and are now firmly associated to both mitochondria and redox homeostasis...
February 24, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29520721/anacardic-acids-from-cashew-nuts-prevent-behavioral-changes-and-oxidative-stress-induced-by-rotenone-in-a-rat-model-of-parkinson-s-disease
#16
Cybelle Façanha Barreto Medeiros-Linard, Belmira Lara da Silveira Andrade-da-Costa, Ricielle Lopes Augusto, Adriana Sereniki, Maria Teresa Sales Trevisan, Renata de Cássia Ribas Perreira, Francisco Thiago Correia de Souza, Glauber Ruda Feitoza Braz, Claudia Jacques Lagranha, Ivone Antônia de Souza, Almir Gonçalves Wanderley, Soraya S Smailli, Simone Sette Lopes Lafayette
Anacardic acids (AAs) are alkyl phenols mainly presenting in cashew nuts. The antioxidant effects of these compounds have been an area of interest in recent research, with findings suggesting potential therapeutic use for certain diseases. Nevertheless, none of these studies were performed in order to test the hypothesis of whether anacardic acids are capable of preventing behavioral changes and oxidative stress induced by the pesticide rotenone in experimental model of Parkinson's disease. In our research, adult male rats were treated orally with AAs (1, 3, 10, 25, 50, or 100 mg/kg/day) 1 h before rotenone (3 mg/kg; s...
March 8, 2018: Neurotoxicity Research
https://www.readbyqxmd.com/read/29511163/wolfram-syndrome-mams-connection
#17
REVIEW
Benjamin Delprat, Tangui Maurice, Cécile Delettre
Wolfram syndrome (WS) is a rare neurodegenerative disease, the main pathological hallmarks of which associate with diabetes, optic atrophy, and deafness. Other symptoms may be identified in some but not all patients. Prognosis is poor, with death occurring around 35 years of age. To date, no treatment is available. WS was first described as a mitochondriopathy. However, the localization of the protein on the endoplasmic reticulum (ER) membrane challenged this hypothesis. ER contacts mitochondria to ensure effective Ca2+ transfer, lipids transfer, and apoptosis within stabilized and functionalized microdomains, termed "mitochondria-associated ER membranes" (MAMs)...
March 6, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29497364/vulnerable-parkin-loss-of-function-drosophila-dopaminergic-neurons-have-advanced-mitochondrial-aging-mitochondrial-network-loss-and-transiently-reduced-autophagosome-recruitment
#18
Juliana Cackovic, Susana Gutierrez-Luke, Gerald B Call, Amber Juba, Stephanie O'Brien, Charles H Jun, Lori M Buhlman
Selective degeneration of substantia nigra dopaminergic (DA) neurons is a hallmark pathology of familial Parkinson's disease (PD). While the mechanism of degeneration is elusive, abnormalities in mitochondrial function and turnover are strongly implicated. An Autosomal Recessive-Juvenile Parkinsonism (AR-JP) Drosophila melanogaster model exhibits DA neurodegeneration as well as aberrant mitochondrial dynamics and function. Disruptions in mitophagy have been observed in parkin loss-of-function models, and changes in mitochondrial respiration have been reported in patient fibroblasts...
2018: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/29497039/er-mitochondria-signaling-in-parkinson-s-disease
#19
REVIEW
Patricia Gómez-Suaga, José M Bravo-San Pedro, Rosa A González-Polo, José M Fuentes, Mireia Niso-Santano
Mitochondria form close physical contacts with a specialized domain of the endoplasmic reticulum (ER), known as the mitochondria-associated membrane (MAM). This association constitutes a key signaling hub to regulate several fundamental cellular processes. Alterations in ER-mitochondria signaling have pleiotropic effects on a variety of intracellular events resulting in mitochondrial damage, Ca2+ dyshomeostasis, ER stress and defects in lipid metabolism and autophagy. Intriguingly, many of these cellular processes are perturbed in neurodegenerative diseases...
March 1, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29491385/mitochondria-associated-membranes-in-aging-and-senescence-structure-function-and-dynamics
#20
REVIEW
Justyna Janikiewicz, Jędrzej Szymański, Dominika Malinska, Paulina Patalas-Krawczyk, Bernadeta Michalska, Jerzy Duszyński, Carlotta Giorgi, Massimo Bonora, Agnieszka Dobrzyn, Mariusz R Wieckowski
Sites of close contact between mitochondria and the endoplasmic reticulum (ER) are known as mitochondria-associated membranes (MAM) or mitochondria-ER contacts (MERCs), and play an important role in both cell physiology and pathology. A growing body of evidence indicates that changes observed in the molecular composition of MAM and in the number of MERCs predisposes MAM to be considered a dynamic structure. Its involvement in processes such as lipid biosynthesis and trafficking, calcium homeostasis, reactive oxygen species production, and autophagy has been experimentally confirmed...
February 28, 2018: Cell Death & Disease
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