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Mitochondrial disease

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https://www.readbyqxmd.com/read/29454114/mitochondria-targeting-mitochondrial-reactive-oxygen-species-with-mitochondriotropic-polyphenolic-based-antioxidants
#1
José Teixeira, Cláudia M Deus, Fernanda Borges, Paulo J Oliveira
Mitochondrial function and regulation of redox balance is fundamental in controlling cellular life and death pathways. Antioxidants have been used to counteract disruption of redox networks, normally associated with progressive loss of cell homeostasis and disease pathophysiology, although therapeutic success is limited mainly due to pharmacokinetic drawbacks. Attempts to improve mitochondrial function in a range of diseases spurred active drug discovery efforts. Currently, the most effective strategy to deliver drugs to mitochondria is the covalent link of lipophilic cations to the bioactive compound...
February 14, 2018: International Journal of Biochemistry & Cell Biology
https://www.readbyqxmd.com/read/29454073/the-accumulation-of-assembly-intermediates-of-the-mitochondrial-complex-i-matrix-arm-is-reduced-by-limiting-glucose-uptake-in-a-neuronal-like-model-of-melas-syndrome
#2
Guillaume Geffroy, Rayane Benyahia, Samuel Frey, Valerie Desquiret-Dumas, Naig Gueguen, Celine Bris, Sophie Belal, Aurore Inisan, Aurelie Renaud, Arnaud Chevrollier, Daniel Henrion, Dominique Bonneau, Franck Letournel, Guy Lenaers, Pascal Reynier, Vincent Procaccio
Ketogenic diet (KD) which combined carbohydrate restriction and the addition of ketone bodies has emerged as an alternative metabolic intervention used as an anticonvulsant therapy or to treat different types of neurological or mitochondrial disorders including MELAS syndrome. MELAS syndrome is a severe mitochondrial disease mainly due to the m.3243A > G mitochondrial DNA mutation. The broad success of KD is due to multiple beneficial mechanisms with distinct effects of very low carbohydrates and ketones...
February 14, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29453932/neurotrophin-3-restores-synaptic-plasticity-in-the-striatum-of-a-mouse-model-of-huntington-s-disease
#3
Victor G Gómez-Pineda, Francisco M Torres-Cruz, César I Vivar-Cortés, Elizabeth Hernández-Echeagaray
AIMS: Neurotrophin-3 (NT-3) is expressed in the mouse striatum; however, it is not clear the NT-3 role in striatal physiology. The expression levels of mRNAs and immune localization of the NT-3 protein and its receptor TrkC are altered in the striatum following damage induced by an in vivo treatment with 3-nitropropionic acid (3-NP), a mitochondrial toxin used to mimic the histopathological hallmarks of Huntington's disease (HD). The aim of this study was to evaluate the role of NT-3 on corticostriatal synaptic transmission and its plasticity in both the control and damaged striatum...
February 17, 2018: CNS Neuroscience & Therapeutics
https://www.readbyqxmd.com/read/29453889/mitochondrial-dysfunction-and-axon-degeneration-in-progressive-multiple-sclerosis
#4
REVIEW
Graham Campbell, Don Mahad
The neuron is the target of inflammatory demyelinating processes in multiple sclerosis (MS). In progressive MS, however, there is a gathering body of evidence indicating that molecular changes converge on mitochondria within neuronal cell bodies. The most reproducible change relates to mitochondrial respiratory chain complex deficiency, which compromises the capacity of neurons to generate ATP. The resulting energy failure state is coupled with an increase in demand for energy by the demyelinated axon, being particularly relevant to the long tracts such as corticospinal tracts with long projection axons...
February 17, 2018: FEBS Letters
https://www.readbyqxmd.com/read/29453877/significance-of-mitochondria-on-cardiometabolic-syndromes
#5
REVIEW
Nair Anupama, G Sindhu, K G Raghu
Metabolic syndromes (MS) are a cluster of disorders like obesity, hypertension, dyslipidemia, and diabetes. Cardiometabolic syndrome (CMS), a branch of MS, is a group of diseases affecting cardiovascular, renal, metabolic, prothrombotic and inflammatory abnormalities due to defects in energy metabolism. Since the emergence of molecular biology and the discovery of pathogenic mitochondrial DNA defect in the 1980s, research advances has revealed a number of common human disease involving mitochondrial dysfunction...
February 17, 2018: Fundamental & Clinical Pharmacology
https://www.readbyqxmd.com/read/29453568/a-method-for-in-vivo-induction-and-ultrastructural-detection-of-mitophagy-in-sertoli-cells
#6
Nabil Eid, Yuko Ito, Akio Horibe, Hitomi Hamaoka, Yoichi Kondo
An emerging body of evidences based on in vitro studies indicate that mitophagy (selective autophagic clearance of damaged mitochondria) is a prosurvival mechanism, specifically under exposure to various stressors. Sertoli cells (SCs) play essential roles in maintenance of spermatogenesis via paracrine interactions with germ cells and other somatic cells in the testis; however, studies investigating mitophagy in SCs are still very few. In this chapter, we give a brief review of mechanisms and detection methods of mitophagy in SCs based on our recent publications on animal models of ethanol toxicity and current literature...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29453449/functional-analysis-of-slc39a8-mutations-and-their-implications-for-manganese-deficiency-and-mitochondrial-disorders
#7
Eun-Kyung Choi, Trang-Tiffany Nguyen, Neil Gupta, Shigeki Iwase, Young Ah Seo
SLC39A8 encodes ZIP8, a divalent metal ion transporter. Mutations in the SLC39A8 gene are associated with congenital disorder of glycosylation type II and Leigh syndrome. Notably, affected patients with both disorders exhibited severe manganese (Mn) deficiency. The cellular function of human SLC39A8 (hSLC39A8) and the mechanisms by which mutations in this protein lead to human diseases are unclear. Herein, we show that hSLC39A8 mediates 54 Mn uptake by the cells, and its expression is regulated by Mn. While expression of wild-type hSLC39A8 increased 54 Mn uptake activity, disease-associated mutations abrogated the ability of the transporter to mediate Mn uptake into the cells, thereby providing a causal link to severe Mn deficiency...
February 16, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29451807/coenzyme-q-10-from-bench-to-clinic-in-aging-diseases-a-translational-review
#8
Francisco M Gutierrez-Mariscal, Elena M Yubero-Serrano, Jose M Villalba, Jose Lopez-Miranda
Coenzyme Q 10 (CoQ 10 ) is a ubiquitous molecule present in all eukaryotic organisms whose principal role in the cell is related to its participation in the electron transport chain in the inner mitochondrial membrane. CoQ 10 plays a major role in the control of cell redox status, and both the amount and functionality of this molecule have been related to the regulation of reactive oxygen species generation. Numerous reports can be found discussing the implications of CoQ 10 supplementation in human studies and clinical trials related to aging...
February 16, 2018: Critical Reviews in Food Science and Nutrition
https://www.readbyqxmd.com/read/29451361/development-of-a-focused-library-of-triazole-linked-privileged-structure-based-conjugates-leading-to-the-discovery-of-novel-phenotypic-hits-against-protozoan-parasitic-infections
#9
Elisa Uliassi, Lorna Piazzi, Federica Belluti, Andrea Mazzanti, Marcel Kaiser, Reto Brun, Carolina B Moraes, Lucio H Freitas-Junior, Sheraz Gul, Maria Kuzikov, Bernhard Ellinger, Chiara Borsari, Maria Paola Costi, Maria Laura Bolognesi
Protozoan infections caused by Plasmodium, Leishmania, and Trypanosoma spp. contribute significantly to the burden of infectious diseases worldwide, causing severe morbidity and mortality. The inadequacy of available treatments calls for cost- and time-effective drug discovery endeavors. To this end, we envisaged the triazole linkage of privileged structures as an effective drug design strategy to generate a focused library of high-quality compounds. The versatility of this approach was combined with the feasibility of a phenotypic assay, integrated with early ADME-tox profiling...
February 16, 2018: ChemMedChem
https://www.readbyqxmd.com/read/29451200/modulation-of-mitochondrial-bioenergetics-as-a-therapeutic-strategy-in-alzheimer-s-disease
#10
REVIEW
Isaac G Onyango
Alzheimer's disease (AD) is an increasingly pressing worldwide public-health, social, political and economic concern. Despite significant investment in multiple traditional therapeutic strategies that have achieved success in preclinical models addressing the pathological hallmarks of the disease, these efforts have not translated into any effective disease-modifying therapies. This could be because interventions are being tested too late in the disease process. While existing therapies provide symptomatic and clinical benefit, they do not fully address the molecular abnormalities that occur in AD neurons...
January 2018: Neural Regeneration Research
https://www.readbyqxmd.com/read/29450720/diabetic-kidney-disease-is-there-a-role-for-glycemic-variability
#11
REVIEW
Savitha Subramanian, Irl B Hirsch
PURPOSE OF REVIEW: Diabetes is the leading cause of kidney disease globally. Diabetic kidney disease (DKD) is a heterogeneous disorder manifested as albuminuria and/or decreasing GFR. Hyperglycemic burden is the major contributor to the development of DKD. In this article, we review the evidence for the contribution of glycemic variability and the pitfalls associated with use of hemoglobin A1c (A1C), the gold standard for assessment of glucose control, in the setting of DKD. RECENT FINDINGS: Glycemic variability, characterized by swings in blood glucose levels, can result in generation of mitochondrial reactive oxygen species, a putative inciting factor for hyperglycemia-induced alterations in intracellular metabolic pathways...
February 15, 2018: Current Diabetes Reports
https://www.readbyqxmd.com/read/29450137/liver-and-metformin-lessons-of-a-fructose-diet-in-mice
#12
Iara Karise, Fernanda Ornellas, Sandra Barbosa-da-Silva, Cristiane Matsuura, Mariano Del Sol, Marcia Barbosa Aguila, Carlos A Mandarim-de-Lacerda
Studies show that the continuous consumption of fructose can lead to nonalcoholic fatty liver disease (NAFLD) and steatohepatitis. We aimed to investigate the role of Metformin in an animal model of liver injury caused by fructose intake, focusing on the molecular markers of lipogenesis, beta-oxidation, and antioxidant defenses. Male three months old C57BL/6 mice were divided into control group (C) and fructose group (F, 47% fructose), maintained for ten weeks. After, the groups received Metformin or vehicle for a further eight weeks: control (C), control + Metformin (CM), fructose (F), and fructose + Metformin (FM)...
June 2017: Biochimie Open
https://www.readbyqxmd.com/read/29449774/reactive-oxygen-species-a-key-constituent-in-cancer-survival
#13
REVIEW
Seema Kumari, Anil Kumar Badana, Murali Mohan G, Shailender G, RamaRao Malla
Background: Cancer is one of the major heterogeneous disease with high morbidity and mortality with poor prognosis. Elevated levels of reactive oxygen species (ROS), alteration in redox balance, and deregulated redox signaling are common hallmarks of cancer progression and resistance to treatment. Mitochondria contribute mainly in the generation of ROS during oxidative phosphorylation. Elevated levels of ROS have been detected in cancers cells due to high metabolic activity, cellular signaling, peroxisomal activity, mitochondrial dysfunction, activation of oncogene, and increased enzymatic activity of oxidases, cyclooxygenases, lipoxygenases, and thymidine phosphorylases...
2018: Biomarker Insights
https://www.readbyqxmd.com/read/29449647/the-pro-inflammatory-phenotype-of-the-human-non-classical-monocyte-subset-is-attributed-to-senescence
#14
Siew-Min Ong, Eva Hadadi, Truong-Minh Dang, Wei-Hseun Yeap, Crystal Tze-Ying Tan, Tze-Pin Ng, Anis Larbi, Siew-Cheng Wong
Human primary monocytes comprise a heterogeneous population that can be classified into three subsets based on CD14 and CD16 expression: classical (CD14 high /CD16 - ), intermediate (CD14 high /CD16 + ), and non-classical (CD14 low /CD16 + ). The non-classical monocytes are the most pro-inflammatory in response to TLR stimulation in vitro, yet they express a remarkably high basal level of miR-146a, a microRNA known to negatively regulate the TLR pathway. This concurrence of a pro-inflammatory status and a high miR-146a level has been associated with cellular senescence in other cell types...
February 15, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29449364/diabetic-cardiomyopathy-an-update-of-mechanisms-contributing-to-this-clinical-entity
#15
REVIEW
Guanghong Jia, Michael A Hill, James R Sowers
Heart failure and related morbidity and mortality are increasing at an alarming rate, in large part, because of increases in aging, obesity, and diabetes mellitus. The clinical outcomes associated with heart failure are considerably worse for patients with diabetes mellitus than for those without diabetes mellitus. In people with diabetes mellitus, the presence of myocardial dysfunction in the absence of overt clinical coronary artery disease, valvular disease, and other conventional cardiovascular risk factors, such as hypertension and dyslipidemia, has led to the descriptive terminology, diabetic cardiomyopathy...
February 16, 2018: Circulation Research
https://www.readbyqxmd.com/read/29449072/merrf-classification-implications-for-diagnosis-and-clinical-trials
#16
REVIEW
Josef Finsterer, Sinda Zarrouk-Mahjoub, John M Shoffner
BACKGROUND: Given the etiologic heterogeneity of disease classification using clinical phenomenology, we employed contemporary criteria to classify variants associated with myoclonic epilepsy with ragged-red fibers (MERRF) syndrome and to assess the strength of evidence of gene-disease associations. Standardized approaches are used to clarify the definition of MERRF, which is essential for patient diagnosis, patient classification, and clinical trial design. METHODS: Systematic literature and database search with application of standardized assessment of gene-disease relationships using modified Smith criteria and of variants reported to be associated with MERRF using modified Yarham criteria...
December 13, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/29448286/-mitochondrial-dysfunctions-and-role-of-coenzyme-q10-in-patients-with-glaucoma
#17
Carl Erb, Katarzyna Konieczka
Mitochondrial function is closely linked to numerous aspects of eye health. Imbalance between the creation of energy and the development of reactive oxygen species (ROS) seems to be the cause of the development of mitochondrial dysfunctions. As a result of this energy deficit, the level of oxidative stress in the eye tissues increases, leading to numerous ophthalmic impairments. It is important to distinguish between primary mitochondrial eye diseases and secondary mitochondrial changes. Primary mitochondrial eye diseases, for example Leber's hereditary optic atrophy (LHON), retinitis pigmentosa and chronic progressive external ophthalmoplegia are caused by direct damage to mitochondrial function induced by defective genes, either located on mitochondrial DNA (mtDNA) or the DNA of the nucleus (nDNA)...
February 2018: Klinische Monatsblätter Für Augenheilkunde
https://www.readbyqxmd.com/read/29448087/assessment-of-mitochondrial-function-following-short-and-long-term-exposure-of-human-bronchial-epithelial-cells-to-total-particulate-matter-from-a-candidate-modified-risk-tobacco-product-and-reference-cigarettes
#18
Dominika Malińska, Jędrzej Szymański, Paulina Patalas-Krawczyk, Bernadeta Michalska, Aleksandra Wojtala, Monika Prill, Małgorzata Partyka, Karolina Drabik, Jarosław Walczak, Alain Sewer, Stephanie Johne, Karsta Luettich, Manuel C Peitsch, Julia Hoeng, Jerzy Duszyński, Joanna Szczepanowska, Marco van der Toorn, Mariusz R Wieckowski
Mitochondrial dysfunction caused by cigarette smoke is involved in the oxidative stress-induced pathology of airway diseases. Reducing the levels of harmful and potentially harmful constituents by heating rather than combusting tobacco may reduce mitochondrial changes that contribute to oxidative stress and cell damage. We evaluated mitochondrial function and oxidative stress in BEAS-2B human bronchial epithelial cells following 1- and 12-week exposures to total particulate matter (TPM) from the aerosol of a candidate modified-risk tobacco product, the Tobacco Heating System 2...
February 12, 2018: Food and Chemical Toxicology
https://www.readbyqxmd.com/read/29448021/risk-assessment-of-silica-nanoparticles-on-liver-injury-in-metabolic-syndrome-mice-induced-by-fructose
#19
Jianmei Li, Xiwei He, Yang Yang, Mei Li, Chenke Xu, Rong Yu
This study aims to assess the effects and the mechanisms of silica nanoparticles (SiNPs) on hepatotoxicity in both normal and metabolic syndrome mouse models induced by fructose. Here, we found that SiNPs exposure lead to improved insulin resistance in metabolic syndrome mice, but markedly worsened hepatic ballooning, inflammation infiltration, and fibrosis. Moreover, SiNPs exposure aggravated liver injury in metabolic syndrome mice by causing serious DNA damage. Following SiNPs exposure, liver superoxide dismutase and catalase activities in metabolic syndrome mice were stimulated, which is accompanied by significantly increased malondialdehyde and 8-hydroxy-2-deoxyguanosine levels as compared to normal mice...
February 12, 2018: Science of the Total Environment
https://www.readbyqxmd.com/read/29447366/parent-of-origin-dependent-gene-expression-in-male-and-female-schistosome-parasites
#20
Julien Kincaid-Smith, Marion A L Picard, Céline Cosseau, Jérôme Boissier, Dany Severac, Christoph Grunau, Eve Toulza
Schistosomes are the causative agents of schistosomiasis, a Neglected Tropical Disease affecting over 230 million people worldwide. Additionally to their major impact on human health, they are also models of choice in evolutionary biology. These parasitic flatworms are unique among the common hermaphroditic trematodes as they have separate sexes. This so-called « evolutionary scandal » displays a female heterogametic genetic sex-determination system (ZZ males and ZW females), as well as a pronounced adult sexual dimorphism...
February 12, 2018: Genome Biology and Evolution
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