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Mitochondrial diseases

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https://www.readbyqxmd.com/read/29156373/mitochondria-targeted-ubiquinone-mitoq-enhances-acetaldehyde-clearance-by-reversing-alcohol-induced-posttranslational-modification-of-aldehyde-dehydrogenase-2-a-molecular-mechanism-of-protection-against-alcoholic-liver-disease
#1
Liuyi Hao, Qian Sun, Wei Zhong, Wenliang Zhang, Xinguo Sun, Zhanxiang Zhou
Alcohol metabolism in the liver generates highly toxic acetaldehyde. Breakdown of acetaldehyde by aldehyde dehydrogenase 2 (ALDH2) in the mitochondria consumes NAD(+) and generates reactive oxygen/nitrogen species, which represents a fundamental mechanism in the pathogenesis of alcoholic liver disease (ALD). A mitochondria-targeted lipophilic ubiquinone (MitoQ) has been shown to confer greater protection against oxidative damage in the mitochondria compared to untargeted antioxidants. The present study aimed to investigate if MitoQ could preserve mitochondrial ALDH2 activity and speed up acetaldehyde clearance, thereby protects against ALD...
November 11, 2017: Redox Biology
https://www.readbyqxmd.com/read/29156129/small-molecule-mitochondrial-uncouplers-and-their-therapeutic-potential
#2
Elizabeth S Childress, Stephanie J Alexopoulos, Kyle L Hoehn, Webster L Santos
Small molecule mitohondrial uncouplers transport protons from the mitochondrial inner membrane space into the mitochondrial matrix independent of ATP synthase, uncoupling nutrient metabolism from ATP generation. The therapeutic potential of mitochondrial uncouplers have been investigated for the treatment metabolic diseases such as obesity and type 2 diabetes (T2D), as well as for neurodegenerative diseases including Alzheimer's disease and Parkinson's disease. This perspective will review the mitochondrial uncouplers reported to date and explore their potential as therapeutics...
November 20, 2017: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/29156062/surgical-technique-for-pulled-in-two-syndrome-three-cases-with-chronic-progressive-external-ophthalmoplegia
#3
Alexander Matthew Jones, Julia Starte, Hamish Dunn, Kate Ahmad, Kimberley Tan
The authors describe three examples of "pulled in two syndrome" (PITS) from a series of 13 patients undergoing strabismus surgery with underlying chronic progressive external ophthalmoplegia (CPEO) and illustrate techniques for recovery of the "pulled in two" extraocular muscle should the complication arise. In all cases, a rectus muscle snapped under minimal tension while held on a strabismus hook during strabismus surgery. Two patients suffered from CPEO as a result of genetic mitochondrial disease, whereas one resulted from presumed mitochondrial toxicity induced by HAART...
November 17, 2017: Journal of Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/29155328/mitochondrial-trna-leu-uur-c3275t-trna-gln-t4363c-and-trna-lys-a8343g-mutations-may-be-associated-with-pcos-and-metabolic-syndrome
#4
Yu Ding, Bo-Hou Xia, Cai-Juan Zhang, Guang-Chao Zhuo
Polycystic ovary syndrome (PCOS) is a very prevalent endocrine disease affecting reproductive women. Clinically, patients with this disorder are more vulnerable to develop type 2 diabetes mellitus (T2DM), cardiovascular events, as well as metabolic syndrome (MetS). To date, the molecular mechanism underlying PCOS remains largely unknown. Previously, we showed that mitochondrial dysfunction caused by mitochondrial DNA (mtDNA) mutation was an important cause for PCOS. In the current study, we described the clinical and biochemical features of a three-generation pedigree with maternally transmitted MetS, combined with PCOS...
November 16, 2017: Gene
https://www.readbyqxmd.com/read/29155101/type-i-ifn-related-netosis-in-ataxia-telangiectasia-and-artemis-deficiency
#5
Ersin Gul, Esra Hazar Sayar, Bilgi Gungor, Fehime Kara Eroglu, Naz Surucu, Sevgi Keles, Sukru Nail Guner, Sıddıka Fındık, Esin Alpdündar, Ihsan Cihan Ayanoglu, Basak Kayaoglu, Busra Nur Geçkin, Hatice Asena Sanli, Tamer Kahraman, Cengiz Yakicier, Meltem Muftuoglu, Berna Oguz, Deniz Nazire Cagdas Ayvaz, Ihsan Gursel, Seza Ozen, Ismail Reisli, Mayda Gursel
BACKGROUND: Pathological inflammatory syndromes of unknown etiology are commonly observed in Ataxia telangiectasia (AT) and Artemis deficiency. Similar inflammatory manifestations also exist in STING-associated vasculopathy in infancy (SAVI) patients. OBJECTIVE: To test the hypothesis that the inflammation associated manifestations observed in AT and Artemis deficient patients stem from increased type I IFN signature leading to neutrophil mediated pathological damage...
November 15, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29154964/lipotoxicity-and-the-gut-liver-axis-in-nash-pathogenesis
#6
REVIEW
Fabio Marra, Gianluca Svegliati-Baroni
The pathogenesis of nanoalcoholic fatty liver disease is still incompletely understood, in particolar the mechanisms whereby a minority of patients develop a more severe phenotype characterized by hepatocellular damage, inflammation, and fibrosis. In this review we discuss two pivotal aspects of the pathogenesis of NASH. We first analyze the initial mechanisms responsible of hepatocellular damage and inflammation, which derive from the toxic effects of excess lipids. Accumulating data indicate that the total amount of triglycerides stored in hepatocytes is not the major determinant of lipotoxicity, and that specific lipid classes act as damaging agents on liver cells...
November 14, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/29154935/ghrelin-alleviates-paclitaxel-induced-peripheral-neuropathy-by-reducing-oxidative-stress-and-enhancing-mitochondrial-anti-oxidant-functions-in-mice
#7
Nobuyuki Ishii, Hironobu Tsubouchi, Ayako Miura, Shigehisa Yanagi, Hiroaki Ueno, Kazutaka Shiomi, Masamitsu Nakazato
Paclitaxel is an effective chemotherapeutic agent, but has some treatment-limiting adverse effects that markedly decrease patients' quality of life. Peripheral neuropathy is one of these, and no treatment for it has been established yet. Ghrelin, an endogenous ligand for the growth hormone secretagogue receptor, is secreted from the stomach and has widespread effects on multiple systems. We investigated the pharmacological potential of ghrelin in preventing paclitaxel-induced peripheral neuropathy using wild-type mice, ghrelin-null mice, and growth hormone secretagogue receptor-null mice...
November 15, 2017: European Journal of Pharmacology
https://www.readbyqxmd.com/read/29154879/perillyl-alcohol-alleviates-amyloid-%C3%AE-peptides-induced-mitochondrial-dysfunction-and-cytotoxicity-in-sh-sy5y-cells
#8
Mohammad Faraz Zafeer, Fakiha Firdaus, Faraz Ahmad, Rizwan Ullah, Ehraz Anis, Mohd Waseem, Asif Ali, Mohammad Mobarak Hossain
Alzheimer's disease (AD) is a progressive neurodegenerative disorder and the most common type of dementia in elderly ( >65years of age). Excessive extra cellular deposits of amyloid beta (Aβ) are a pathological feature of AD. Aβ can cause cell death through oxidative damage; recent studies have implicated opening of mPTP as a detrimental event in AD-related mitochondrial dysfunctions. Over the past few years, natural compounds with antioxidant properties have shown promise for intervention in AD.
November 14, 2017: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/29154852/the-mitochondrial-uncoupling-protein-2-gene-is-causal-for-the-spontaneous-polycystic-liver-diseases-in-mice
#9
Misa Hirose, Paul Schilf, Sarah Rohde, Yask Gupta, Tiphaine Sancerni, Marie-Clotilde Alves-Guerra, Christian Sina, Robert Jaster, Bruno Miroux, Saleh M Ibrahim
Polycystic liver diseases (PCLDs) are autosomal dominant disorders. To date, 3 genes are known to be associated with the disease, SEC63 and PRKCSH and LRP5. Here, we report that mice deficient in the mitochondrial uncoupling protein 2 gene (Ucp2(-/-)) spontaneously developed PCLDs when they were over 12months old. Macroscopical observation, blood chemistry as well as histopathological analysis demonstrated the PCLDs found in Ucp2(-/-) mice were very similar to the findings in human PCLDs. This is the first report describing the gene encoding mitochondrial protein is causative for PCLDs...
November 14, 2017: Mitochondrion
https://www.readbyqxmd.com/read/29154270/ubisol-q10-a-nanomicellar-water-soluble-formulation-of-coq10-treatment-inhibits-alzheimer-type-behavioral-and-pathological-symptoms-in-a-double-transgenic%C3%A2-mouse%C3%A2-tgapeswe-%C3%A2-psen1de9-model-of-alzheimer-s-disease
#10
Krithika Muthukumaran, Annie Kanwar, Caleb Vegh, Alexandra Marginean, Austin Elliott, Nicholas Guilbeault, Alexander Badour, Marianna Sikorska, Jerome Cohen, Siyaram Pandey
 Alzheimer's disease (AD) is one of the most common neurodegenerative pathologies for which there are no effective therapies to halt disease progression. Given the increase in the incidence of this disorder, there is an urgent need for pharmacological intervention. Unfortunately, recent clinical trials produced disappointing results. Molecular mechanisms of AD are converging on the notion that mitochondrial dysfunction, oxidative stress, and accumulation of dysfunctional proteins are involved in AD pathology...
November 16, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29154141/novel-genes-associated-with-amyotrophic-lateral-sclerosis-diagnostic-and-clinical-implications
#11
REVIEW
Ruth Chia, Adriano Chiò, Bryan J Traynor
BACKGROUND: The disease course of amyotrophic lateral sclerosis (ALS) is rapid and, because its pathophysiology is unclear, few effective treatments are available. Genetic research aims to understand the underlying mechanisms of ALS and identify potential therapeutic targets. The first gene associated with ALS was SOD1, identified in 1993 and, by early 2014, more than 20 genes had been identified as causative of, or highly associated with, ALS. These genetic discoveries have identified key disease pathways that are therapeutically testable and could potentially lead to the development of better treatments for people with ALS...
November 16, 2017: Lancet Neurology
https://www.readbyqxmd.com/read/29152896/trimetazidine-restores-the-positive-adaptation-to-exercise-training-by-mitigating-statin-induced-skeletal-muscle-injury
#12
Ming Song, Fang-Fang Chen, Yi-Hui Li, Lei Zhang, Feng Wang, Ran-Ran Qin, Zhi-Hao Wang, Ming Zhong, Meng-Xiong Tang, Wei Zhang, Lu Han
BACKGROUND: Exercise rehabilitation is demonstrated to improve the prognosis of patients with coronary heart disease (CHD). Statins, as the key medicine to lower cholesterol in CHD, result in skeletal muscle injury and impair exercise training adaptation. Energy metabolism dysfunction is identified as the potential mechanism underlying statin-induced skeletal muscle injury. In this study, we investigated the effects of the metabolic modulator trimetazidine on skeletal muscle energy metabolism and statin-associated exercise intolerance...
November 19, 2017: Journal of Cachexia, Sarcopenia and Muscle
https://www.readbyqxmd.com/read/29152251/oxygen-the-forgotten-nutrient
#13
Paul Trayhurn
O2 is essential for the maintenance and growth of aerobic animals, similar to the essentiality of what are classically considered nutrients. Nevertheless, O2 is not customarily regarded as a nutrient, this reflecting the route by which it enters the body - through the lungs or gills in vertebrates, rather than via the mouth and gastrointestinal tract. A relative deficiency of O2 occurs at high altitudes and during deep-sea diving, to which distinct adaptations occur. Deficiency is also evident in lung diseases such as emphysema...
2017: Journal of Nutritional Science
https://www.readbyqxmd.com/read/29151958/santamarine-inhibits-nf-%C3%B0%C2%BAb-and-stat3-activation-and-induces-apoptosis-in-hepg2-liver-cancer-cells-via-oxidative-stress
#14
Tahir Mehmood, Amara Maryam, Xiangge Tian, Muhammad Khan, Tonghui Ma
Sesquiterpene lactones have long been used in traditional Chinese medicines to treat inflammatory diseases. Recently, sesquiterpene lactone family compounds have been recognized as potential anticancer agents. Thus, it is necessary to explore new sesquiterpene lactones and their antitumor mechanism for cancer treatments. In the present study, we have explored the potential anti-cancer activity of a novel sesquiterpene lactone compound "santamarine" (STM) in HepG2 cells. It inhibited proliferation and induced apoptosis dose-dependently with IC50 ~ 70 μM...
2017: Journal of Cancer
https://www.readbyqxmd.com/read/29151191/silica-nanoparticle-exposure-inducing-granulosa-cell-apoptosis-and-follicular-atresia-in-female-balb-c-mice
#15
Jianhui Liu, Man Yang, Li Jing, Lihua Ren, Jialiu Wei, Jin Zhang, Feng Zhang, Junchao Duan, Xianqing Zhou, Zhiwei Sun
Given that the effects of ultrafine fractions (< 0.1 μm) on reproductive diseases are gaining attention, this study aimed to explore the influence of silica nanoparticle (SiNP)-induced female reproductive dysfunction. In this study, 80 female mice were randomly divided into four groups including a control group and three concentrations of SiNP groups (7, 21, 35 mg/kg). Mice were exposed to the vehicle control and silica nanoparticles by tracheal perfusion every 3 days a total of five times in 15 days...
November 19, 2017: Environmental Science and Pollution Research International
https://www.readbyqxmd.com/read/29151169/parkinson-s-disease-experimental-models-and-reality
#16
REVIEW
Peizhou Jiang, Dennis W Dickson
Parkinson's disease (PD) is a chronic, progressive movement disorder of adults and the second most common neurodegenerative disease after Alzheimer's disease. Neuropathologic diagnosis of PD requires moderate-to-marked neuronal loss in the ventrolateral substantia nigra pars compacta and α-synuclein (αS) Lewy body pathology. Nigrostriatal dopaminergic neurodegeneration correlates with the Parkinsonian motor features, but involvement of other peripheral and central nervous system regions leads to a wide range of non-motor features...
November 18, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/29150527/transcriptional-regulators-of-redox-balance-and-other-homeostatic-processes-with-the-potential-to-alter-neurodegenerative-disease-trajectory
#17
REVIEW
Scott W Burnside, Giles E Hardingham
Diverse neurodegenerative diseases share some common aspects to their pathology, with many showing evidence of disruption to the brain's numerous homeostatic processes. As such, imbalanced inflammatory status, glutamate dyshomeostasis, hypometabolism and oxidative stress are implicated in many disorders. That these pathological processes can influence each other both up- and downstream makes for a complicated picture, but means that successfully targeting one area may have an effect on others. This targeting requires an understanding of the mechanisms by which homeostasis is maintained during health, in order to uncover strategies to boost homeostasis in disease...
November 17, 2017: Biochemical Society Transactions
https://www.readbyqxmd.com/read/29150293/a-unique-and-promising-combination-of-medications-for-the-treatment-of-alzheimer-s-disease
#18
James D Weinstein
At present there is no therapy for Alzheimer's Disease which completely stops the progressive dementia effecting late onset Alzheimer's Disease (AD) patients. It is felt that the main reason for this failure is that AD appears to be a disease caused by four major pathological processes. To date, efforts to develop treatments have addressed only one or another of these four etiologies. However, even a partially effective therapy against one cause allows the others, untreated, to continue their inexorable destruction of the neurons of the brain...
November 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/29149835/forkhead-transcription-factors-formulating-a-foxo-target-for-cognitive-loss
#19
Kenneth Maiese
BACKGROUND: With almost 47 million individuals worldwide suffering from some aspect of dementia, it is clear that cognitive loss impacts a significant proportion of the global population. Unfortunately, definitive treatments to resolve or prevent the onset of cognitive loss are limited. In most cases such care is currently non-existent prompting the need for novel treatment strategies. METHODS: Mammalian forkhead transcription factors of the O class (FoxO) are one such avenue of investigation that offer an exciting potential to bring new treatments forward for disorders that involve cognitive loss...
November 15, 2017: Current Neurovascular Research
https://www.readbyqxmd.com/read/29149768/mitochondrial-dna-changes-in-pedunculopontine-cholinergic-neurons-in-parkinson-s
#20
Alexander G Bury, Angela Pyle, Joanna L Elson, Laura Greaves, Christopher M Morris, Gavin Hudson, Ilse S Pienaar
In Parkinson's disease (PD), mitochondrial dysfunction associates with nigral dopaminergic neuronal loss. Cholinergic neuronal loss co-occurs, particularly within a brainstem structure, the pedunculopontine nucleus (PPN). We isolated single cholinergic neurons from post-mortem PPNs of aged controls and PD patients. Mitochondrial DNA (mtDNA) copy number and mtDNA deletions were increased significantly in PD patients compared to controls. Furthermore, compared to controls the PD patients had significantly more PPN cholinergic neurons containing mtDNA deletion levels exceeding 60%, a level associated with deleterious effects on oxidative phosphorylation...
November 17, 2017: Annals of Neurology
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