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Mitochondrial disease complex I

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https://www.readbyqxmd.com/read/27911893/the-mitochondrial-m-aaa-protease-prevents-demyelination-and-hair-greying
#1
Shuaiyu Wang, Julie Jacquemyn, Sara Murru, Paola Martinelli, Esther Barth, Thomas Langer, Carien M Niessen, Elena I Rugarli
The m-AAA protease preserves proteostasis of the inner mitochondrial membrane. It ensures a functional respiratory chain, by controlling the turnover of respiratory complex subunits and allowing mitochondrial translation, but other functions in mitochondria are conceivable. Mutations in genes encoding subunits of the m-AAA protease have been linked to various neurodegenerative diseases in humans, such as hereditary spastic paraplegia and spinocerebellar ataxia. While essential functions of the m-AAA protease for neuronal survival have been established, its role in adult glial cells remains enigmatic...
December 2016: PLoS Genetics
https://www.readbyqxmd.com/read/27899525/reactive-oxygen-species-induce-virus-independent-mavs-oligomerization-in-systemic-lupus-erythematosus
#2
Iwona A Buskiewicz, Theresa Montgomery, Elizabeth C Yasewicz, Sally A Huber, Michael P Murphy, Richard C Hartley, Ryan Kelly, Mary K Crow, Andras Perl, Ralph C Budd, Andreas Koenig
The increased expression of genes induced by type I interferon (IFN) is characteristic of viral infections and systemic lupus erythematosus (SLE). We showed that mitochondrial antiviral signaling (MAVS) protein, which normally forms a complex with retinoic acid gene I (RIG-I)-like helicases during viral infection, was activated by oxidative stress independently of RIG-I helicases. We found that chemically generated oxidative stress stimulated the formation of MAVS oligomers, which led to mitochondrial hyperpolarization and decreased adenosine triphosphate production and spare respiratory capacity, responses that were not observed in similarly treated cells lacking MAVS...
November 29, 2016: Science Signaling
https://www.readbyqxmd.com/read/27882077/potential-protective-effects-of-autophagy-activated-in-mpp-treated-astrocytes
#3
Cunzhou Shen, Wenbiao Xian, Hongyan Zhou, Ling Chen, Zhong Pei
Astrocytes, which have various important functions, have previously been associated with Parkinsons disease (PD), particularly in 1-methyl-4-phenylpyridinium (MPP+) and 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) models of PD. MPP+ is the toxic metabolite of MPTP and is generated by the enzymatic activity of monoamine oxidase B, which is predominantly located in astrocytes. MPP+ acts as a mitochondrial complex I inhibitor. Autophagy is an evolutionarily conserved self-digestion pathway in eukaryotic cells, which occurs in response to various types of stress, including starvation and oxidative stress...
November 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/27871794/accessory-subunit-nuym-ndufs4-is-required-for-stability-of-the-electron-input-module-and-activity-of-mitochondrial-complex-i
#4
Flora Kahlhöfer, Katarzyna Kmita, Ilka Wittig, Klaus Zwicker, Volker Zickermann
Mitochondrial complex I is an intricate 1MDa membrane protein complex with a central role in aerobic energy metabolism. The minimal form of complex I consists of fourteen central subunits that are conserved from bacteria to man. In addition, eukaryotic complex I comprises some 30 accessory subunits of largely unknown function. The gene for the accessory NDUFS4 subunit of human complex I is a hot spot for fatal pathogenic mutations in humans. We have deleted the gene for the orthologous NUYM subunit in the aerobic yeast Yarrowia lipolytica, an established model system to study eukaryotic complex I and complex I linked diseases...
November 18, 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27867381/a-critical-reappraisal-of-neutrophil-extracellular-traps-and-netosis-mimics-based-on-differential-requirements-for-protein-citrullination
#5
Maximilian F Konig, Felipe Andrade
NETosis, an antimicrobial form of neutrophil cell death, is considered a primary source of citrullinated autoantigens in rheumatoid arthritis (RA) and immunogenic DNA in systemic lupus erythematosus (SLE). Activation of the citrullinating enzyme peptidylarginine deiminase type 4 (PAD4) is believed to be essential for neutrophil extracellular trap (NET) formation and NETosis. PAD4 is therefore viewed as a promising therapeutic target to inhibit the formation of NETs in both diseases. In this review, we examine the evidence for PAD4 activation during NETosis and provide experimental data to suggest that protein citrullination is not a universal feature of NETs...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27848206/tanshinone-i-attenuates-the-effects-of-a-challenge-with-h2o2-on-the-functions-of-tricarboxylic-acid-cycle-and-respiratory-chain-in-sh-sy5y-cells
#6
Marcos Roberto de Oliveira, Cristina Ribas Fürstenau, Izabel Cristina Custódio de Souza, Gustavo da Costa Ferreira
Tanshinone I (T-I; C18H12O3) is a cytoprotective molecule. T-I has been viewed as an antioxidant and anti-inflammatory agent exerting neuroprotective actions in several experimental models. Nonetheless, the mechanisms underlying the beneficial effects of T-I in mammalian cells are not completely understood yet. Mitochondrial dysfunction has been associated with several neurodegenerative diseases which remain uncured. Therefore, there is increasing interest in compounds that may be used in the prevention or treatment of those pathologies...
November 15, 2016: Molecular Neurobiology
https://www.readbyqxmd.com/read/27847310/dual-loss-of-succinate-dehydrogenase-sdh-and-complex-i-activity-is-necessary-to-recapitulate-the-metabolic-phenotype-of-sdh-mutant-tumors
#7
Doriane Lorendeau, Gianmarco Rinaldi, Ruben Boon, Pieter Spincemaille, Kristine Metzger, Christian Jäger, Stefan Christen, Xiangyi Dong, Sabine Kuenen, Karin Voordeckers, Patrik Verstreken, David Cassiman, Pieter Vermeersch, Catherine Verfaillie, Karsten Hiller, Sarah-Maria Fendt
Mutations in succinate dehydrogenase (SDH) are associated with tumor development and neurodegenerative diseases. Only in tumors, loss of SDH activity is accompanied with the loss of complex I activity. Yet, it remains unknown whether the metabolic phenotype of SDH mutant tumors is driven by loss of complex I function, and whether this contributes to the peculiarity of tumor development versus neurodegeneration. We addressed this question by decoupling loss of SDH and complex I activity in cancer cells and neurons...
November 12, 2016: Metabolic Engineering
https://www.readbyqxmd.com/read/27843288/leber-s-hereditary-optic-neuropathy-is-multiorgan-not-mono-organ
#8
REVIEW
Josef Finsterer, Sinda Zarrouk-Mahjoub
Leber's hereditary optic neuropathy (LHON) is a maternally inherited mitochondrial disorder with bilateral loss of central vision primarily due to mitochondrial DNA (mtDNA) mutations in subunits of complex I in the respiratory chain (primary LHON mutations), while other mtDNA mutations can also be causative. Since the first description, it is known that LHON is not restricted to the eyes but is a multisystem disorder additionally involving the central nervous system, ears, endocrinological organs, heart, bone marrow, arteries, kidneys, or the peripheral nervous system...
2016: Clinical Ophthalmology
https://www.readbyqxmd.com/read/27836895/lifelong-quercetin-enrichment-and-cardioprotection-in-mdx-utrn-ice
#9
Christopher Ballmann, Thomas Denney, Ronald J Beyers, Tiffany S Quindry, Matthew Romero, Rajesh H Amin, Joshua T Selsby, John C Quindry
Duchenne Muscular Dystrophy (DMD) is associated with progressive cardiac pathology, however, the SIRT1/PGC1-α activator quercetin may cardioprotect dystrophic hearts. We tested the extent to which long term 0.2% dietary quercetin enrichment attenuates dystrophic cardiopathology in Mdx/Utrn(+/-) mice. At 2 months, Mdx/Utrn(+/-) mice were fed quercetin enriched (Mdx/Utrn(+/-)-Q) or control diet (Mdx/Utrn(+/-)) for 8 months. Control C57BL/10 (C57) animals were fed a control diet for 10 months. Cardiac function was quantified by MRI at 2 and 10 months...
November 11, 2016: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/27828948/control-of-mitochondrial-function-and-cell-growth-by-the-atypical-cadherin-fat1
#10
Longyue L Cao, Dario F Riascos-Bernal, Prameladevi Chinnasamy, Charlene M Dunaway, Rong Hou, Mario A Pujato, Brian P O'Rourke, Veronika Miskolci, Liang Guo, Louis Hodgson, Andras Fiser, Nicholas E S Sibinga
Mitochondrial products such as ATP, reactive oxygen species, and aspartate are key regulators of cellular metabolism and growth. Abnormal mitochondrial function compromises integrated growth-related processes such as development and tissue repair, as well as homeostatic mechanisms that counteract ageing and neurodegeneration, cardiovascular disease, and cancer. Physiologic mechanisms that control mitochondrial activity in such settings remain incompletely understood. Here we show that the atypical Fat1 cadherin acts as a molecular 'brake' on mitochondrial respiration that regulates vascular smooth muscle cell (SMC) proliferation after arterial injury...
November 9, 2016: Nature
https://www.readbyqxmd.com/read/27826939/a-novel-iron-chelator-radical-scavenger-ameliorates-motor-dysfunction-and-improves-life-span-and-mitochondrial-biogenesis-in-sod1-g93a-als-mice
#11
Sagit Golko-Perez, Tamar Amit, Orit Bar-Am, Moussa B H Youdim, Orly Weinreb
The aim of the present study was to evaluate the therapeutic effect of the novel neuroprotective multitarget brain permeable monoamine oxidase inhibitor/iron chelating-radical scavenging drug, VAR10303 (VAR), co-administered with high-calorie/energy-supplemented diet (ced) in SOD1(G93A) transgenic amyotrophic lateral sclerosis (ALS) mice. Administration of VAR-ced was initiated after the appearance of disease symptoms (at day 88), as this regimen is comparable with the earliest time at which drug therapy could start in ALS patients...
November 8, 2016: Neurotoxicity Research
https://www.readbyqxmd.com/read/27815040/the-addition-of-ketone-bodies-alleviates-mitochondrial-dysfunction-by-restoring-complex-i-assembly-in-a-melas-cellular-model
#12
Samuel Frey, Guillaume Geffroy, Valerie Desquiret-Dumas, Naig Gueguen, Celine Bris, Sophie Belal, Patrizia Amati-Bonneau, Arnaud Chevrollier, Magalie Barth, Daniel Henrion, Guy Lenaers, Dominique Bonneau, Pascal Reynier, Vincent Procaccio
Ketogenic Diet used to treat refractory epilepsy for almost a century may represent a treatment option for mitochondrial disorders for which effective treatments are still lacking. Mitochondrial complex I deficiencies are involved in a broad spectrum of inherited diseases including Mitochondrial Encephalomyopathy, Lactic Acidosis and Stroke-like episodes syndrome leading to recurrent cerebral insults resembling strokes and associated with a severe complex I deficiency caused by mitochondrial DNA (mtDNA) mutations...
November 1, 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27814651/mitochondria-in-the-pathophysiology-of-alzheimer-s-and-parkinson-s-diseases
#13
Isaac G Onyango, Shaharyar M Khan, James P Bennett
Mitochondria are responsible for the majority of energy production in energy-intensive tissues like brain, modulate Ca(+2) signaling and control initiation of cell death. Because of their extensive use of oxygen and lack of protective histone proteins, mitochondria are vulnerable to oxidative stress (ROS)-induced damage to their genome (mtDNA), respiratory chain proteins and ROS repair enzymes. Animal and cell models of PD use toxins that impair mitochondrial complex I activity. Maintenance of mitochondrial mass, mitochondrial biogenesis (mitobiogenesis), particularly in high-energy brain, occurs through complex signaling pathways involving the upstream "master regulator" PGC-1alpha that is transcriptionally and post-translationally regulated...
January 1, 2017: Frontiers in Bioscience (Landmark Edition)
https://www.readbyqxmd.com/read/27806193/neuroprotective-effect-of-a-new-7-8-dihydroxycoumarin-based-fe2-cu2-chelator-in-cell-and-animal-models-of-parkinson-s-disease
#14
Pabla Aguirre, Olimpo García-Beltrán, Victoria Tapia, Yorka Muñoz, Bruce Kennedy Cassels, Marco T Nunez
Disturbed iron homeostasis, often coupled to mitochondrial dysfunction, plays an important role in the progression of common neurodegenerative diseases such as Parkinson disease (PD). Recent studies have underlined the relevance of iron chelation therapy for the treatment of these diseases. Here we describe the synthesis, chemical and biological characterization of the multifunctional chelator 7,8-dihydroxy-4-((methylamino)methyl)-2H-chromen-2-one (DHC12). Metal selectivity of DHC12 was Cu2+ ̴ Fe2+ > Zn2+ > Fe3+...
November 2, 2016: ACS Chemical Neuroscience
https://www.readbyqxmd.com/read/27805009/metformin-uniquely-prevents-thrombosis-by-inhibiting-platelet-activation-and-mtdna-release
#15
Guang Xin, Zeliang Wei, Chengjie Ji, Huajie Zheng, Jun Gu, Limei Ma, Wenfang Huang, Susan L Morris-Natschke, Jwu-Lai Yeh, Rui Zhang, Chaoyi Qin, Li Wen, Zhihua Xing, Yu Cao, Qing Xia, Yanrong Lu, Ke Li, Hai Niu, Kuo-Hsiung Lee, Wen Huang
Thrombosis and its complications are the leading cause of death in patients with diabetes. Metformin, a first-line therapy for type 2 diabetes, is the only drug demonstrated to reduce cardiovascular complications in diabetic patients. However, whether metformin can effectively prevent thrombosis and its potential mechanism of action is unknown. Here we show, metformin prevents both venous and arterial thrombosis with no significant prolonged bleeding time by inhibiting platelet activation and extracellular mitochondrial DNA (mtDNA) release...
November 2, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27802911/mitochondrial-multifaceted-dysfunction-in-schizophrenia-complex-i-as-a-possible-pathological-target
#16
REVIEW
Dorit Ben-Shachar
Mitochondria are key players in various essential cellular processes beyond being the main energy supplier of the cell. Accordingly, they are involved in neuronal synaptic transmission, neuronal growth and sprouting and consequently neuronal plasticity and connectivity. In addition, mitochondria participate in the modulation of gene transcription and inflammation as well in physiological responses in health and disease. Schizophrenia is currently regarded as a neurodevelopmental disorder associated with impaired immune system, aberrant neuronal differentiation and abnormalities in various neurotransmitter systems mainly the dopaminergic, glutaminergic and GABAergic...
October 29, 2016: Schizophrenia Research
https://www.readbyqxmd.com/read/27801955/curcumin-prevents-mitochondrial-dynamics-disturbances-in-early-5-6-nephrectomy-relation-to-oxidative-stress-and-mitochondrial-bioenergetics
#17
Omar Emiliano Aparicio-Trejo, Edilia Tapia, Eduardo Molina-Jijón, Omar Noel Medina-Campos, Norma Angélica Macías-Ruvalcaba, Juan Carlos León-Contreras, Rogelio Hernández-Pando, Fernando E García-Arroyo, Magdalena Cristóbal, Laura Gabriela Sánchez-Lozada, José Pedraza-Chaverri
Five-sixths nephrectomy (5/6NX) is a widely used model to study the mechanisms leading to renal damage in chronic kidney disease (CKD). However, early alterations on renal function, mitochondrial dynamics, and oxidative stress have not been explored yet. Curcumin is an antioxidant that has shown nephroprotection in 5/6NX-induced renal damage. The aim of this study was to explore the effect of curcumin on early mitochondrial alterations induced by 5/6NX in rats. In isolated mitochondria, 5/6NX-induced hydrogen peroxide production was associated with decreased activity of complexes I and V, decreased activity of antioxidant enzymes, alterations in oxygen consumption and increased MDA-protein adducts...
November 1, 2016: BioFactors
https://www.readbyqxmd.com/read/27793643/nuclear-but-not-mitochondrial-encoded-oxphos-genes-are-altered-in-aging-mild-cognitive-impairment-and-alzheimer-s-disease
#18
Diego Mastroeni, Omar M Khdour, Elaine Delvaux, Jennifer Nolz, Gary Olsen, Nicole Berchtold, Carl Cotman, Sidney M Hecht, Paul D Coleman
INTRODUCTION: We have comprehensively described the expression profiles of mitochondrial DNA and nuclear DNA genes that encode subunits of the respiratory oxidative phosphorylation (OXPHOS) complexes (I-V) in the hippocampus from young controls, age matched, mild cognitively impaired (MCI), and Alzheimer's disease (AD) subjects. METHODS: Hippocampal tissues from 44 non-AD controls (NC), 10 amnestic MCI, and 18 AD cases were analyzed on Affymetrix Hg-U133 plus 2...
October 25, 2016: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/27785568/novel-mutations-in-iba57-are-associated-with-leukodystrophy-and-variable-clinical-phenotypes
#19
Alessandra Torraco, Anna Ardissone, Federica Invernizzi, Teresa Rizza, Giuseppe Fiermonte, Marcello Niceta, Nadia Zanetti, Diego Martinelli, Angelo Vozza, Daniela Verrigni, Michela Di Nottia, Eleonora Lamantea, Daria Diodato, Marco Tartaglia, Carlo Dionisi-Vici, Isabella Moroni, Laura Farina, Enrico Bertini, Daniele Ghezzi, Rosalba Carrozzo
Defects of the Fe/S cluster biosynthesis represent a subgroup of diseases affecting the mitochondrial energy metabolism. In the last years, mutations in four genes (NFU1, BOLA3, ISCA2 and IBA57) have been related to a new group of multiple mitochondrial dysfunction syndromes characterized by lactic acidosis, hyperglycinemia, multiple defects of the respiratory chain complexes, and impairment of four lipoic acid-dependent enzymes: α-ketoglutarate dehydrogenase complex, pyruvic dehydrogenase, branched-chain α-keto acid dehydrogenase complex and the H protein of the glycine cleavage system...
October 26, 2016: Journal of Neurology
https://www.readbyqxmd.com/read/27781077/neuroprotective-effects-of-a%C3%A3-a%C3%A3-euterpe-oleracea-mart-against-rotenone-in-vitro-exposure
#20
Alencar Kolinski Machado, Ana Cristina Andreazza, Tatiane Morgana da Silva, Aline Augusti Boligon, Vanusa do Nascimento, Gustavo Scola, Angela Duong, Francine Carla Cadoná, Euler Esteves Ribeiro, Ivana Beatrice Mânica da Cruz
Neuropsychiatric diseases, such as bipolar disorder (BD) and schizophrenia (SCZ), have a very complex pathophysiology. Several current studies describe an association between psychiatric illness and mitochondrial dysfunction and consequent cellular modifications, including lipid, protein, and DNA damage, caused by cellular oxidative stress. Euterpe oleracea (açaí) is a powerful antioxidant fruit. Açaí is an Amazonian palm fruit primarily found in the lowlands of the Amazonian rainforest, particularly in the floodplains of the Amazon River...
2016: Oxidative Medicine and Cellular Longevity
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