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https://www.readbyqxmd.com/read/29027761/oxidative-stress-and-mitochondrial-dynamics-malfunction-are-linked-in-pelizaeus-merzbacher-disease
#1
Montserrat Ruiz, Mélina Bégou, Nathalie Launay, Pablo Ranea-Robles, Patrizia Bianchi, Jone López-Erauskin, Laia Morató, Cristina Guilera, Bérengère Petit, Catherine Vaurs-Barriere, Céline Guéret-Gonthier, Marie-Noëlle Bonnet-Dupeyron, Stéphane Fourcade, Johan Auwerx, Odile Boespflug-Tanguy, Aurora Pujol
Pelizaeus-Merzbacher disease (PMD) is a fatal hypomyelinating disorder characterized by early impairment of motor development, nystagmus, choreoathetotic movements, ataxia and progressive spasticity. PMD is caused by variations in the proteolipid protein gene PLP1, which encodes the two major myelin proteins of the central nervous system, PLP and its spliced isoform DM20, in oligodendrocytes. Large duplications including the entire PLP1 gene are the most frequent causative mutation leading to the classical form of PMD...
October 13, 2017: Brain Pathology
https://www.readbyqxmd.com/read/29023512/the-ultrastructural-characteristics-of-porcine-hepatocytes-donated-after-cardiac-death-and-preserved-with-warm-machine-perfusion-preservation
#2
Hiroki Bochimoto, Naoto Matsuno, Yo Ishihara, Tatsuya Shonaka, Daisuke Koga, Yoshiki Hira, Yuji Nishikawa, Hiroyuki Furukawa, Tsuyoshi Watanabe
The effects of warm machine perfusion preservation of liver grafts donated after cardiac death on the intracellular three-dimensional ultrastructure of the organelles in hepatocytes remain unclear. Here we analyzed comparatively the ultrastructure of the endomembrane systems in porcine hepatocytes under warm ischemia and successive hypothermic and midthermic machine perfusion preservation, a type of the warm machine perfusion. Porcine liver grafts which had a warm ischemia time of 60 minutes were perfused for 4 hours with modified University of Wisconsin gluconate solution...
2017: PloS One
https://www.readbyqxmd.com/read/29021915/optical-coherence-tomography-findings-in-methanol-toxicity
#3
Kendra A Klein, Alexis K Warren, Caroline R Baumal, Thomas R Hedges
BACKGROUND: Methanol toxicity poses a significant public health problem in developing countries, and in Southeast Asia, where the most common source of poisoning is via adulterated liquor in local drinks. Methanol toxicity can have devastating visual consequences and retinal specialists should be aware of the features of this toxic optic neuropathy. The authors report a case of severe systemic methanol toxicity and relatively mild optic neuropathy demonstrating unique retinal changes on optical coherence tomography (OCT)...
2017: International Journal of Retina and Vitreous
https://www.readbyqxmd.com/read/29019354/iron-sulfur-cluster-biosynthesis-and-trafficking-impact-on-human-disease-conditions
#4
C Wachnowsky, I Fidai, J A Cowan
Iron-sulfur clusters (Fe-S) are one of the most ancient, ubiquitous and versatile classes of metal cofactors found in nature. Proteins that contain Fe-S clusters constitute one of the largest families of proteins, with varied functions that include electron transport, regulation of gene expression, substrate binding and activation, radical generation, and, more recently discovered, DNA repair. Research during the past two decades has shown that mitochondria are central to the biogenesis of Fe-S clusters in eukaryotic cells via a conserved cluster assembly machinery (ISC assembly machinery) that also controls the synthesis of Fe-S clusters of cytosolic and nuclear proteins...
October 11, 2017: Metallomics: Integrated Biometal Science
https://www.readbyqxmd.com/read/29019267/ge11-peptide-conjugated-selenium-nanoparticles-for-egfr-targeted-oridonin-delivery-to-achieve-enhanced-anticancer-efficacy-by-inhibiting-egfr-mediated-pi3k-akt-and-ras-raf-mek-erk-pathways
#5
Jiang Pi, Jinhuan Jiang, Huaihong Cai, Fen Yang, Hua Jin, Peihui Yang, Jiye Cai, Zheng W Chen
Selenium nanoparticles (Se NPs) have attracted increasing interest in recent decades because of their anticancer, immunoregulation, and drug carrier functions. In this study, GE11 peptide-conjugated Se NPs (GE11-Se NPs), a nanosystem targeting EGFR over-expressed cancer cells, were synthesized for oridonin delivery to achieve enhanced anticancer efficacy. Oridonin loaded and GE11 peptide conjugated Se NPs (GE11-Ori-Se NPs) were found to show enhanced cellular uptake in cancer cells, which resulted in enhanced cancer inhibition against cancer cells and reduced toxicity against normal cells...
November 2017: Drug Delivery
https://www.readbyqxmd.com/read/28994715/%C3%AE-amyloid-and-the-pathomechanisms-of-alzheimer-s-disease-a-comprehensive-view
#6
REVIEW
Botond Penke, Ferenc Bogár, Lívia Fülöp
Protein dyshomeostasis is the common mechanism of neurodegenerative diseases such as Alzheimer's disease (AD). Aging is the key risk factor, as the capacity of the proteostasis network declines during aging. Different cellular stress conditions result in the up-regulation of the neurotrophic, neuroprotective amyloid precursor protein (APP). Enzymatic processing of APP may result in formation of toxic Aβ aggregates (β-amyloids). Protein folding is the basis of life and death. Intracellular Aβ affects the function of subcellular organelles by disturbing the endoplasmic reticulum-mitochondria cross-talk and causing severe Ca(2+)-dysregulation and lipid dyshomeostasis...
October 10, 2017: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/28993979/methamphetamine-augments-concurrent-astrocyte-mitochondrial-stress-oxidative-burden-and-antioxidant-capacity-tipping-the-balance-in-hiv-associated-neurodegeneration
#7
Kathleen Borgmann, Anuja Ghorpade
Methamphetamine (METH) use, with and without human immunodeficiency virus (HIV)-1 comorbidity, exacerbates neurocognitive decline. Oxidative stress is a probable neurotoxic mechanism during HIV-1 central nervous system infection and METH abuse, as viral proteins, antiretroviral therapy and METH have each been shown to induce mitochondrial dysfunction. However, the mechanisms regulating mitochondrial homeostasis and overall oxidative burden in astrocytes are not well understood in the context of HIV-1 infection and METH abuse...
October 9, 2017: Neurotoxicity Research
https://www.readbyqxmd.com/read/28993562/ultrastructural-features-of-canine-neuroaxonal-dystrophy-in-a-papillon-dog
#8
Miyuu Tanaka, Shinobu Yamaguchi, Hideo Akiyoshi, Masaya Tsuboi, Kazuyuki Uchida, Takeshi Izawa, Jyoji Yamate, Mitsuru Kuwamura
Neuroaxonal dystrophy (NAD) is a neurodegenerative disease characterized by severe axonal swelling (spheroids) throughout the nervous system. In dogs, NAD has been reported in several breeds and a missense mutation in PLA2G6 gene has recently been identified in the Papillon dog NAD. Here we performed ultrastructural analysis to clarify the detailed ultrastructural features of the Papillon dog NAD. Dystrophic axons consisted of accumulation of filamentous materials, tubulovesicular structures, and swollen edematous mitochondria with degenerated inner membranes were often observed in the CNS...
October 10, 2017: Journal of Veterinary Medical Science
https://www.readbyqxmd.com/read/28993273/acute-mental-stress-induces-mitochondrial-bioenergetic-crisis-and-hyper-fission-along-with-aberrant-mitophagy-in-the-gut-mucosa-in-rodent-model-of-stress-related-mucosal-disease
#9
Rudranil De, Somnath Mazumder, Souvik Sarkar, Subhashis Debsharma, Asim Azhar Siddiqui, Shubhra Jyoti Saha, Chinmoy Banerjee, Shiladitya Nag, Debanjan Saha, Uday Bandyopadhyay
Psychological stress, depression and anxiety lead to multiple organ dysfunctions wherein stress-related mucosal disease (SRMD) is common to people experiencing stress and also occur as a side effect in patients admitted to intensive care units; however the underlying molecular aetiology is still obscure. We report that in rat-SRMD model, cold restraint-stress severely damaged gut mitochondrial functions to generate superoxide anion (O2(•-)), depletion of ATP and shifted mitochondrial fission-fusion dynamics towards enhanced fission to induce mucosal injury...
October 6, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28993115/recurrent-exposure-to-ferric-oxide-nanoparticles-alters-myocardial-oxidative-stress-apoptosis-and-necrotic-markers-in-male-mice
#10
Vijayprakash Manickam, Madhivadhani Periyasamy, Vasanth Dhakshinamoorthy, Lakshmikanthan Panneerselvam, Ekambaram Perumal
The cardiotoxicity of iron oxide nanoparticles (Fe2O3-NPs) in mice was investigated. The mice were intraperitoneally administered with Fe2O3-NPs at the dose of 25 and 50 mg/kg bw for 30 days at seven days interval. In vivo MRI analysis reveals the Fe2O3-NPs accumulation in the cardiac system. Also, serum iron estimation and Prussian blue staining confirms the iron deposition in circulatory system. Cardiac dysfunction was assessed by ECG analysis and further validated by evaluating the functional markers such as cardiac Troponin-1 (cTnI) expression, AChE activity and levels of LDH and CK-MB in cardiac tissue...
October 6, 2017: Chemico-biological Interactions
https://www.readbyqxmd.com/read/28991908/mitochondrial-dual-coding-genes-in-trypanosome-brucei
#11
Laura E Kirby, Donna Koslowsky
Trypanosoma brucei is transmitted between mammalian hosts by the tsetse fly. In the mammal, they are exclusively extracellular, continuously replicating within the bloodstream. During this stage, the mitochondrion lacks a functional electron transport chain (ETC). Successful transition to the fly, requires activation of the ETC and ATP synthesis via oxidative phosphorylation. This life cycle leads to a major problem: in the bloodstream, the mitochondrial genes are not under selection and are subject to genetic drift that endangers their integrity...
October 9, 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28990533/blood-brain-barrier-disruption-leads-to-postoperative-cognitive-dysfunction
#12
Bin Wang, Siyuan Li, Xipeng Cao, Xinghui Dou, Jingzhu Li, Ling Wang, Mingshan Wang, Yanlin Bi
BACKGROUND: Postoperative cognitive dysfunction (POCD) has received considerable attention as one of the main postoperative complications. The underlying mechanism of POCD in elderly subjects has not been fully elucidated to date. The central nervous system (CNS) is isolated from the bloodstream by the blood brain barrier (BBB) that consists of endothelial cells, capillary blood vessels and the tight junctions. The tight junctions carry out significant biological functions that are associated with the CNS and the blood circulation...
October 9, 2017: Current Neurovascular Research
https://www.readbyqxmd.com/read/28989672/photochromic-coenzyme-q-derivatives-switching-redox-potentials-with-light
#13
Nadja A Simeth, Andrea C Kneuttinger, Reinhard Sterner, Burkhard König
Coenzyme Q is an important redox cofactor involved in a variety of cellular processes, and is thus found in several cell compartments. We report a photochromic derivative of coenzyme Q that combines the molecular structures of the redox active cofactor and a photochromic dye. Light irradiation triggers an electronic rearrangement reversibly changing the redox potential. We used this effect to control the intermolecular redox reaction of the photochromic coenzyme Q derivative with dihydropyridine in solution by light irradiation...
September 1, 2017: Chemical Science
https://www.readbyqxmd.com/read/28987512/genes-linking-mitochondrial-function-cognitive-impairment-and-depression-are-associated-with-endophenotypes-serving-precision-medicine
#14
Peter Petschner, Xenia Gonda, Daniel Baksa, Nora Eszlari, Michael Trivaks, Gabriella Juhasz, Gyorgy Bagdy
Mitochondria densely populate cells in central nervous system providing essential energy for neurons and influencing synaptic plasticity. Harm to these organelles can impair cognitive performance through damaged neurotransmission and altered Ca(2+) homeostasis. Impaired cognition could be one underlying factor which can characterize major depressive disorder, a huge burden for society marked by depressed mood and anhedonia. A growing body of evidence binds mitochondrial dysfunctions with the disease. Cognitive disturbances with different severity are also observable in several patients, suggesting that damage or inherited alterations of mitochondria may have an important role in depression...
October 5, 2017: Neuroscience
https://www.readbyqxmd.com/read/28986232/rifampicin-inhibits-rotenone-induced-microglial-inflammation-via-enhancement-of-autophagy
#15
Yanran Liang, Tianen Zhou, Ying Chen, Danyu Lin, Xiuna Jing, Sudan Peng, Dezhi Zheng, Zhifen Zeng, Ming Lei, Xia Wu, Kaixun Huang, Lianhong Yang, Songhua Xiao, Jun Liu, Enxiang Tao
Mitochondrial and autophagic dysfunction, as well as neuroinflammation, are associated with the pathophysiology of Parkinson's disease (PD). Rotenone, an inhibitor of mitochondrial complex I, has been associated as an environmental neurotoxin related to PD. Our previous studies reported that rifampicin inhibited microglia activation and production of proinflammatory mediators induced by rotenone, but the precise mechanism has not been completely elucidated. BV2 cells were pretreated for 2h with rifampicin followed by 0...
October 3, 2017: Neurotoxicology
https://www.readbyqxmd.com/read/28986095/modulation-of-mitochondrial-dynamics-by-treadmill-training-to-improve-gait-and-mitochondrial-deficiency-in-a-rat-model-of-parkinson-s-disease
#16
Chieh-Sen Chuang, Jui-Chih Chang, Fu-Chou Cheng, Ko-Hung Liu, Hong-Lin Su, Chin-San Liu
PURPOSE: Parkinson's disease (PD) is a progressive degenerative central nervous system disorder that particularly impairs motor function. As PD advances, gait disorders become more pronounced and are often difficult to treat with current pharmacological therapies. Physical activity improves both mobility in and the daily living activities of patients with PD. Mitochondrial alterations and oxidative stress contribute to PD progression. Therefore, the association between mitochondria and exercise in PD and the implicated regulation of mitochondrial proteins was explored in this study...
October 3, 2017: Life Sciences
https://www.readbyqxmd.com/read/28984064/loss-of-sirt2-leads-to-axonal-degeneration-and-locomotor-disability-associated-with-redox-and-energy-imbalance
#17
Stéphane Fourcade, Laia Morató, Janani Parameswaran, Montserrat Ruiz, Tatiana Ruiz-Cortés, Mariona Jové, Alba Naudí, Paloma Martínez-Redondo, Mara Dierssen, Isidre Ferrer, Francesc Villarroya, Reinald Pamplona, Alejandro Vaquero, Manel Portero-Otín, Aurora Pujol
Sirtuin 2 (SIRT2) is a member of a family of NAD(+) -dependent histone deacetylases (HDAC) that play diverse roles in cellular metabolism and especially for aging process. SIRT2 is located in the nucleus, cytoplasm, and mitochondria, is highly expressed in the central nervous system (CNS), and has been reported to regulate a variety of processes including oxidative stress, genome integrity, and myelination. However, little is known about the role of SIRT2 in the nervous system specifically during aging. Here, we show that middle-aged, 13-month-old mice lacking SIRT2 exhibit locomotor dysfunction due to axonal degeneration, which was not present in young SIRT2 mice...
October 5, 2017: Aging Cell
https://www.readbyqxmd.com/read/28983470/the-espf-n-terminal-of-enterohemorrhagic-escherichia-coli-o157-h7-edl933w-imparts-stronger-toxicity-effects-on-ht-29-cells-than-the-c-terminal
#18
Xiangyu Wang, Yanli Du, Ying Hua, Muqing Fu, Cong Niu, Bao Zhang, Wei Zhao, Qiwei Zhang, Chengsong Wan
Enterohemorrhagic Escherichia coli (EHEC) O157:H7 EspF is an important multifunctional protein that destroys the tight junctions of intestinal epithelial cells and promotes host cell apoptosis. However, its molecular mechanism remains elusive. We knocked out the espF sequence (747 bp, ΔespF), N-terminal sequence (219 bp, ΔespFN ), and C-terminal sequence (528 bp, ΔespFC ) separately using the pKD46-mediated λ Red homologous recombination system. Then, we built the corresponding complementation strains, namely, ΔespF/pespF, ΔespFN/pespFN , and ΔespFC/pespFC by overlap PCR, which were used in infecting HT-29 cells and BALB/C mice...
2017: Frontiers in Cellular and Infection Microbiology
https://www.readbyqxmd.com/read/28982845/mitochondrial-dysfunction-and-redox-imbalance-as-a-diagnostic-marker-of-free-radical-diseases
#19
REVIEW
Ekaterina Georgieva, Donika Ivanova, Zhivko Zhelev, Rumiana Bakalova, Maya Gulubova, Ichio Aoki
The intracellular redox balance (redox status) is a dynamic system that may change via many factors. Mitochondria are one of the most important among them. These organelles are the main intracellular source of energy. They are essential for maintaining cellular homeostasis due to regulation of many biochemical processes. The mitochondrial dynamics change during cellular activities and in some cases, can cause an overproduction of reactive oxygen species (ROS), which encourages the induction of oxidative DNA damage and up- or down-regulation of phosphatases, proliferative/anti-proliferative factors, apoptotic/anti-apoptotic factors, etc...
October 2017: Anticancer Research
https://www.readbyqxmd.com/read/28978719/pharmacological-dual-inhibition-of-tumor-and-tumor-induced-functional-limitations-in-transgenic-model-of-breast-cancer
#20
Ruizhong Wang, Poornima Bhat-Nakshatri, Maria B Padua, Mayuri S Prasad, Manjushree Anjanappa, Max Jacobson, Courtney Finnearty, Victoria Sefcsik, Kyle McElyea, Rachael Redmond, George E Sandusky, Narsimha Penthala, Peter A Crooks, Jianguo Liu, Teresa Zimmers, Harikrishna Nakshatri
Breast cancer progression is associated with systemic effects including functional limitations and sarcopenia without the appearance of overt cachexia. Autocrine/paracrine actions of cytokines/chemokines produced by cancer cells mediate cancer progression and functional limitations. The cytokine-inducible transcription factor NF-κB could be central to this process, as it displays oncogenic functions and is integral to the Pax7:MyoD:Pgc-1β:miR-486 myogenesis axis. We tested this possibility using the MMTV-PyMT transgenic mammary tumor model and the NF-κB inhibitor dimethylaminoparthenolide (DMAPT)...
October 4, 2017: Molecular Cancer Therapeutics
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