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https://www.readbyqxmd.com/read/28239650/mitophagy-and-mitochondrial-biogenesis-in-atrial-tissue-of-patients-undergoing-heart-surgery-with-cardiopulmonary-bypass
#1
Allen M Andres, Kyle C Tucker, Amandine Thomas, David J R Taylor, David Sengstock, Salik M Jahania, Reza Dabir, Somayeh Pourpirali, Jamelle A Brown, David G Westbrook, Scott W Ballinger, Robert M Mentzer, Roberta A Gottlieb
Mitophagy occurs during ischemia/reperfusion (I/R) and limits oxidative stress and injury. Mitochondrial turnover was assessed in patients undergoing cardiac surgery involving cardiopulmonary bypass (CPB). Paired biopsies of right atrial appendage before initiation and after weaning from CPB were processed for protein analysis, mitochondrial DNA/nuclear DNA ratio (mtDNA:nucDNA ratio), mtDNA damage, mRNA, and polysome profiling. Mitophagy in the post-CPB samples was evidenced by decreased levels of mitophagy adapters NDP52 and optineurin in whole tissue lysate, decreased Opa1 long form, and translocation of Parkin to the mitochondrial fraction...
February 23, 2017: JCI Insight
https://www.readbyqxmd.com/read/28238805/pharmacological-inhibition-of-lsd1-and-mtor-reduces-mitochondrial-retention-and-associated-ros-levels-in-the-red-blood-cells-of-sickle-cell-disease
#2
Ramasamy Jagadeeswaran, Benjamin A Vazquez, Muthusamy Thiruppathi, Balaji B Ganesh, Vinzon Ibanez, Shuaiying Cui, James D Engel, Alan M Diamond, Robert E Molokie, Joseph DeSimone, Donald Lavelle, Angela Rivers
Sickle cell disease (SCD), an inherited blood disorder caused by a point mutation that renders hemoglobin susceptible to polymerization when deoxygenated, affects millions of people worldwide. Manifestations of SCD include chronic hemolytic anemia, inflammation, painful vaso-occlusive crises, multisystem organ damage, and reduced life expectancy. Part of SCD pathophysiology is the excessive formation of intracellular reactive oxygen species (ROS) in SCD red blood cells (RBCs) which accelerates their hemolysis...
February 23, 2017: Experimental Hematology
https://www.readbyqxmd.com/read/28238795/reversal-of-phenotypic-abnormalities-by-crispr-cas9-mediated-gene-correction-in-huntington-disease-patient-derived-induced-pluripotent-stem%C3%A2-cells
#3
Xiaohong Xu, Yilin Tay, Bernice Sim, Su-In Yoon, Yihui Huang, Jolene Ooi, Kagistia Hana Utami, Amin Ziaei, Bryan Ng, Carola Radulescu, Donovan Low, Alvin Yu Jin Ng, Marie Loh, Byrappa Venkatesh, Florent Ginhoux, George J Augustine, Mahmoud A Pouladi
Huntington disease (HD) is a dominant neurodegenerative disorder caused by a CAG repeat expansion in HTT. Here we report correction of HD human induced pluripotent stem cells (hiPSCs) using a CRISPR-Cas9 and piggyBac transposon-based approach. We show that both HD and corrected isogenic hiPSCs can be differentiated into excitable, synaptically active forebrain neurons. We further demonstrate that phenotypic abnormalities in HD hiPSC-derived neural cells, including impaired neural rosette formation, increased susceptibility to growth factor withdrawal, and deficits in mitochondrial respiration, are rescued in isogenic controls...
February 21, 2017: Stem Cell Reports
https://www.readbyqxmd.com/read/28238714/neuroprotective-effects-of-kukoamine-a-on-neurotoxin-induced-parkinson-s-model-through-apoptosis-inhibition-and-autophagy-enhancement
#4
XiaoLong Hu, Qi Song, Xin Li, DanDan Li, Qiao Zhang, WeiHong Meng, QingChun Zhao
Parkinson's disease (PD) is characterized by the loss of dopaminergic neurons in substantia nigra (SN). Our previous study demonstrated Kukoamine A to exhibit strong neuroprotective effects through anti-oxidative stress, anti-inflamation, anti-excitoxicity. In the present study, MPP(+) and MPTP-induced PD models of cell and animal were used to investigate the effects of KuA on PD. Our results demonstrated that KuA ameliorated cell loss and mitochondrial membrane potential (MMP) loss, and inhibited Bax/Bcl-2 ratio and MAPKs family that were induced by MPP(+)...
February 24, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28238526/mitochondrial-dna-oxidation-induces-imbalanced-activity-of-nlrp3-nlrp6-inflammasomes-by-activation-of-caspase-8-and-brcc36-in-dry-eye
#5
Wei Chi, Xia Hua, Xin Chen, Fang Bian, Xiaoyong Yuan, Lili Zhang, Xiaoran Wang, Ding Chen, Ruzhi Deng, Zhijie Li, Yizhi Liu, Cintia S de Paiva, Stephen C Pflugfelder, De-Quan Li
The concept of innate immunity has been expanded to recognize environmental pathogens other than microbial components. However, whether and how the innate immunity is initiated by epithelium in response to environmental physical challenges such as low humidity and high osmolarity in an autoimmune disease, dry eye, is still largely unknown. Using two experimental dry eye models, primary human corneal epithelial cultures exposed to hyperosmolarity and mouse ocular surface facing desiccating stress, we uncovered novel innate immunity pathway by ocular surface epithelium, where oxidized mitochondrial DNA induces imbalanced activation of NLRP3/NLRP6 inflammasomes via stimulation of caspase-8 and BRCC36 in response to environmental stress...
February 23, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28238458/increased-levels-of-anti-phosphatidylcholine-and-anti-phosphatidylethanolamine-antibodies-in-pediatric-patients-with-cerebral-infarction
#6
Seigo Korematsu, Hiroshi Yamada, Hiroaki Miyahara, Kenji Ihara
Cerebral infarction in children is rare and often occurs secondary to moyamoya disease, hereditary coagulopathies, vasculitis, antiphospholipid antibody syndrome, heart disease, mitochondrial disease. However, in some cases, the causes of cerebral infarction is unknown. In this study, we detected increased levels of serum anti-phosphatidylcholine and anti-phosphatidylethanolamine IgG antibodies in three pediatric patients with cerebral infarction whose primary disorders are unknown by routine examination. For the five disease control patients of cerebral infarction due to other primary disorders, there was no such increase in these antibodies levels...
February 23, 2017: Brain & Development
https://www.readbyqxmd.com/read/28237964/vulnerability-to-oxygen-glucose-deprivation-of-primary-neurons-derived-from-tg2576-alzheimer-mice-role-of-intraneuronal-abeta-accumulation-and-astrocytes
#7
Vito Antonio Baldassarro, Alessandra Marchesini, Luciana Giardino, Laura Calzà
Microvascular dysfunction is considered an integral part of Alzheimer disease (AD) pathogenesis, but the possible relationship between amyloid pathology, microvascular dysfunction and cell death is still unclear. In order to investigate the influence of intraneuronal Abeta (Aβ) accumulation on vulnerability to hypoxia, we isolated primary cortical neurons from Tg2576 (carrying the APPSwe mutation) and Wt fetal mice. We first demonstrated that neurons isolated from Tg2576 new-born mice show an increase in VEGFa mRNA expression and a decrease in the expression of the two VEGF receptors, i...
February 24, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28237843/characterization-of-energy-and-neurotransmitter-metabolism-in-cortical-glutamatergic-neurons-derived-from-human-induced-pluripotent-stem-cells-a-novel-approach-to-study-metabolism-in-human-neurons
#8
Blanca I Aldana, Yu Zhang, Maria Fog Lihme, Lasse K Bak, Jørgen E Nielsen, Bjørn Holst, Poul Hyttel, Kristine K Freude, Helle S Waagepetersen
Alterations in the cellular metabolic machinery of the brain are associated with neurodegenerative disorders such as Alzheimer's disease. Novel human cellular disease models are essential in order to study underlying disease mechanisms. In the present study, we characterized major metabolic pathways in neurons derived from human induced pluripotent stem cells (hiPSC). With this aim, cultures of hiPSC-derived neurons were incubated with [U-(13)C]glucose, [U-(13)C]glutamate or [U-(13)C]glutamine. Isotopic labeling in metabolites was determined using gas chromatography coupled to mass spectrometry, and cellular amino acid content was quantified by high-performance liquid chromatography...
February 22, 2017: Neurochemistry International
https://www.readbyqxmd.com/read/28237732/discerning-the-antioxidant-mechanism-of-rapanone-a-naturally-occurring-benzoquinone-with-iron-complexing-and-radical-scavenging-activities
#9
Karen de la Vega-Hernández, Manuel Antuch, Osmany Cuesta-Rubio, Yanier Núñez-Figueredo, Gilberto L Pardo-Andreu
Oxidative stress resulting from iron and reactive oxygen species (ROS) homeostasis breakdown has been implicated in several diseases. Therefore, molecules capable of binding iron and/or scavenging ROS may be reasonable strategies for protecting cells. Rapanone is a naturally occurring hydroxyl-benzoquinone with a privileged chelating structure. In this work, we addressed the antioxidant properties of rapanone concerning its iron-chelating and scavenging activities, and its protective potential against iron and tert-butyl hydroperoxide-induced damage to mitochondria...
February 20, 2017: Journal of Inorganic Biochemistry
https://www.readbyqxmd.com/read/28237103/automated-analysis-of-fluorescence-colocalization-application-to-mitophagy
#10
A Sauvat, H Zhou, M Leduc, L C Gomes-da-Silva, L Bezu, K Müller, S Forveille, P Liu, L Zhao, G Kroemer, O Kepp
Mitophagy is a peculiar form of organelle-specific autophagy that targets mitochondria. This process ensures cellular homeostasis, as it fosters the disposal of aged and damaged mitochondria that otherwise would be prone to produce reactive oxygen species and hence endanger genomic stability. Similarly, autophagic clearance of depolarized mitochondria plays a fundamental role in organismal homeostasis as exemplified by the link between Parkinson disease and impaired function of the mitophagy-mediating proteins PINK1 and Parkin...
2017: Methods in Enzymology
https://www.readbyqxmd.com/read/28236843/multiple-sclerosis-and-air-pollution-exposure-mechanisms-toward-brain-autoimmunity
#11
Sayed Esmaeil Mousavi, Pouria Heydarpour, Jacques Reis, Masoud Amiri, Mohammad Ali Sahraian
The association between neurodegenerative diseases and environmental exposures, in particular air pollution, has been noticed in the last two decades, but the importance of this environmental factor in multiple sclerosis (MS) pathogenesis has not been considered extensively. However, recent evidence suggests that major mechanisms involved in MS pathogenesis, such as inflammatory factors expression, free radicals overproduction, the blood brain barrier (BBB) breakdown, neuroinflammation, vitamin D deficiency and mitochondrial dysfunction could also occur due to exposure to air pollutants...
March 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28236508/phenotypical-features-of-a-new-dominant-gdap1-pathogenic-variant-p-r226del-in-axonal-charcot-marie-tooth-disease
#12
Tania García-Sobrino, Patricia Blanco-Arias, Francesc Palau, Carmen Espinós, Laura Ramirez, Anna Estela, Beatriz San Millán, Manuel Arias, María-Jesús Sobrido, Julio Pardo
There are few reports on axonal CMT due to dominant GDAP1 mutations. We describe two unrelated Spanish families with a dominant axonal CMT. A novel in frame GAA deletion in exon 5 of the GDAP1 gene (c.677_679del; p.R226del) was identified in both families. Disease onset varied from early childhood to adulthood. Affected family members complained of distal lower limb weakness, cramps and foot deformities with variable CMTNS score in both families. Several individuals were asymptomatic or had paraesthesia only, however neurological examination and nerve conduction studies demonstrated neuropathic signs...
January 17, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28236433/regulators-of-mitochondrial-quality-control-differ-in-subcutaneous-fat-of-metabolically-healthy-and-unhealthy-obese-monkeys
#13
Kylie Kavanagh, Ashley T Davis, Diane E Peters, Andre C LeGrand, Manish S Bharadwaj, Anthony J A Molina
OBJECTIVE: Obesity exists with and without accompanying cardiometabolic disease, termed metabolically unhealthy obesity (MUO) and healthy obesity (MHO), respectively. Underlying differences in the ability of subcutaneous (SQ) fat to respond to nutrient excess are emerging as a key pathway. This study aimed to document the first spontaneous animal model of MHO and MUO and differences in SQ adipose tissue. METHODS: Vervet monkeys (Chlorocebus aethiops; N = 171) were screened for metabolic syndrome...
February 25, 2017: Obesity
https://www.readbyqxmd.com/read/28236380/my-sweetheart-is-broken-role-of-glucose-in-diabetic-cardiomyopathy
#14
REVIEW
Manoja K Brahma, Mark E Pepin, Adam R Wende
Despite overall reductions in heart disease prevalence, the risk of developing heart failure has remained 2-fold greater among people with diabetes. Growing evidence has supported that fluctuations in glucose level and uptake contribute to cardiovascular disease (CVD) by modifying proteins, DNA, and gene expression. In the case of glucose, clinical studies have shown that increased dietary sugars for healthy individuals or poor glycemic control in diabetic patients further increased CVD risk. Furthermore, even after decades of maintaining tight glycemic control, susceptibility to disease progression can persist following a period of poor glycemic control through a process termed "glycemic memory...
February 2017: Diabetes & Metabolism Journal
https://www.readbyqxmd.com/read/28236040/the-oral-administration-of-d-galactose-induces-abnormalities-within-the-mitochondrial-respiratory-chain-in-the-brain-of-rats
#15
Josiane Budni, Michelle Lima Garcez, Francielle Mina, Tatiani Bellettini-Santos, Sabrina da Silva, Aline Pereira da Luz, Gustavo Luiz Schiavo, Hemily Batista-Silva, Giselli Scaini, Emílio Luiz Streck, João Quevedo
D-Galactose (D-gal) chronic administration via intraperitoneal and subcutaneous routes has been used as a model of aging and Alzheimer disease in rodents. Intraperitoneal and subcutaneous administration of D-gal causes memory impairments, a reduction in the neurogenesis of adult mice, an increase in the levels of the amyloid precursor protein and oxidative damage; However, the effects of oral D-gal remain unclear. The aim of this study was to evaluate whether the oral administration of D-gal induces abnormalities within the mitochondrial respiratory chain of rats...
February 24, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28235896/pathogenic-huntington-alters-bmp-signaling-and-synaptic-growth-through-local-disruptions-of-endosomal-compartments
#16
Yulia Akbergenova, J Troy Littleton
Huntington's disease (HD) is a neurodegenerative disorder caused by expansion of a polyglutamine (polyQ) stretch within the Huntingtin (Htt) protein. Pathogenic Htt disrupts multiple neuronal processes, including gene expression, axonal trafficking, proteasome and mitochondrial activity, and intracellular vesicle trafficking. However, the primary pathogenic mechanism and subcellular site of action for mutant Htt are still unclear. Using a Drosophila HD model, we found that pathogenic Htt expression leads to a profound overgrowth of synaptic connections that directly correlates with the levels of Htt at nerve terminals...
February 24, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28235666/identification-of-novel-anti-cancer-agents-by-applying-insilico-methods-for-inhibition-of-tspo-protein
#17
Manan Bhargavi, Sree Kanth Sivan, Sarita Rajender Potlapally
Cancer is a genomic disease characterised as impaired cellular energy metabolism. Cancer cells derive most of their energy from oxidative phosphorylation unlike normal ones during cell progression TSPO protein present in external mitochondrial membrane, is involved in various cellular functions like Cell proliferation, mitochondrial respiration, synthesis of steroids and also participates in import of cholesterol into the inner mitochondrial membrane from outside of the membrane of mitochondria. The 3D model of TSPO protein is built using comparative homology modelling techniques and validated by proSA, Ramachandran plot and ERRAT in the present work...
February 14, 2017: Computational Biology and Chemistry
https://www.readbyqxmd.com/read/28235551/mitophagy-in-neurodegeneration-and-aging
#18
REVIEW
Elayne M Fivenson, Sofie Lautrup, Nuo Sun, Morten Scheibye-Knudsen, Tinna V Stevnsner, Hilde Nilsen, Vilhelm A Bohr, Evandro F Fang
Mitochondrial dysfunction contributes to normal aging and a wide spectrum of age-related diseases, including neurodegenerative disorders such as Parkinson's disease and Alzheimer's disease. It is important to maintain a healthy mitochondrial population which is tightly regulated by proteolysis and mitophagy. Mitophagy is a specialized form of autophagy that regulates the turnover of damaged and dysfunctional mitochondria, organelles that function in producing energy for the cell in the form of ATP and regulating energy homeostasis...
February 21, 2017: Neurochemistry International
https://www.readbyqxmd.com/read/28231545/aqueous-extract-of-cortex-dictamni-protects-h9c2-cardiomyocytes-from-hypoxia-reoxygenation-induced-oxidative-stress-and-apoptosis-by-pi3k-akt-signaling-pathway
#19
Lin Li, Yunfeng Zhou, Yanlin Li, Lili Wang, Lan Sun, Lidong Zhou, Hiderori Arai, Yun Qi, Yang Xu
Ischemia-reperfusion injury is the major manifestation of ischemic heart disease, which facilitates cardiac arrhythmias, heart failure and death. Oxidative stress and apoptosis have been involved in the pathogenesis of myocardial ischemia-reperfusion injury. Modern pharmacological studies have indicated that the extracts and active compounds of Cortex Dictamni exhibit antioxidative and cardiovascular protective activities. This study was designed to investigate the protective effect of aqueous extract of Cortex Dictamni (CDAE) on regulating hypoxia/reoxygenation (H/R)-induced cardiomyocytes oxidative stress and apoptosis...
February 20, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28231026/late-administration-of-a-palladium-lipoic-acid-complex-poly-mva-modifies-cardiac-mitochondria-but-not-functional-or-structural-manifestations-of-radiation-induced-heart-disease-in-a-rat-model
#20
Vijayalakshmi Sridharan, John W Seawright, Francis J Antonawich, Merrill Garnett, Maohua Cao, Preeti Singh, Marjan Boerma
Exposure of the heart to ionizing radiation can cause adverse myocardial remodeling. In small animal models, local heart irradiation causes persistent alterations in cardiac mitochondrial function and swelling. POLY-MVA is a dietary supplement that contains a palladium lipoic acid complex that targets mitochondrial complex I and has been demonstrated to have greater redox potential than lipoic acid alone. POLY-MVA improves mitochondrial function and anti-oxidant enzyme activity in the aged rat heart. In this study, we tested whether POLY-MVA can mitigate cardiac effects of ionizing radiation...
February 23, 2017: Radiation Research
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