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Mitochondrial disease

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https://www.readbyqxmd.com/read/28551810/mitochondria-targeted-antioxidants-for-the-treatment-of-cardiovascular-disorders
#1
Hyoung Kyu Kim, Jin Han
The heart continuously supplies blood to the entire body throughout the lifetime, requiring a huge amount of the bioenergy molecule adenosine triphosphate (ATP). As the major subcellular organs that produce ATP through oxidative phosphorylation and the citrate-synthesis cycle, mitochondria inevitably produce chemically reactive species as byproducts. Those species are known to be major effectors of mitochondrial dysfunction, many of which are involved in various cardiovascular diseases, causing apoptotic/necrotic loss of cardiac myocytes and/or defects in the energy balance within the myocardium...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28551809/mitochondrial-transplantation-in-myocardial-ischemia-and-reperfusion-injury
#2
Borami Shin, Douglas B Cowan, Sitaram M Emani, Pedro J Del Nido, James D McCully
Ischemic heart disease remains the leading cause of death worldwide. Mitochondria are the power plant of the cardiomyocyte, generating more than 95% of the cardiac ATP. Complex cellular responses to myocardial ischemia converge on mitochondrial malfunction which persists and increases after reperfusion, determining the extent of cellular viability and post-ischemic functional recovery. In a quest to ameliorate various points in pathways from mitochondrial damage to myocardial necrosis, exhaustive pharmacologic and genetic tools have targeted various mediators of ischemia and reperfusion injury and procedural techniques without applicable success...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28551808/mitochondrial-heteroplasmy
#3
George B Stefano, Christina Bjenning, Fuzhou Wang, Nan Wang, Richard M Kream
Genetic polymorphisms, in concert with well-characterized etiology and progression of major pathologies, plays a significant role in aberrant processes afflicting human populations. Mitochondrial heteroplasmy represents a dynamically determined co-expression of inherited polymorphisms and somatic pathology in varying ratios within individual mitochondrial DNA (mtDNA) genomes with repetitive patterns of tissue specificity. The ratios of the MtDNA genomes represent a balance between healthy and pathological cellular outcomes...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28551806/mitochondrial-dysfunction-in-the-diabetic-kidney
#4
Kumar Sharma
The role of mitochondria in diabetic complications has been viewed as a source of excess superoxide production leading to cell dysfunction. However, with the lack of benefit of non-specific anti-oxidant approaches this view needs to be re-evaluated. With recent studies using real-time imaging of superoxide, metabolomics, flux studies, transcriptomics and proteomics a new appreciation for the role of mitochondria in the evolution of diabetic kidney disease has emerged. Ongoing studies to further unravel the time course and mechanisms that reduce mitochondrial function will be relevant to novel therapies that could have a major impact on diabetic kidney disease and other diabetic complications...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28551805/mitochondria-damage-and-kidney-disease
#5
Pu Duann, Pei-Hui Lin
The kidney is a vital organ that demands an extraordinary amount of energy to actively maintain the body's metabolism, plasma hemodynamics, electrolytes and water homeostasis, nutrients reabsorption, and hormone secretion. Kidney is only second to the heart in mitochondrial count and oxygen consumption. As such, the health and status of the energy power house, the mitochondria, is pivotal to the health and proper function of the kidney. Mitochondria are heterogeneous and highly dynamic organelles and their functions are subject to complex regulations through modulation of its biogenesis, bioenergetics, dynamics and clearance within cell...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28551803/insulin-resistance-and-mitochondrial-dysfunction
#6
Alba Gonzalez-Franquesa, Mary-Elizabeth Patti
Insulin resistance precedes and predicts the onset of type 2 diabetes (T2D) in susceptible humans, underscoring its important role in the complex pathogenesis of this disease. Insulin resistance contributes to multiple tissue defects characteristic of T2D, including reduced insulin-stimulated glucose uptake in insulin-sensitive tissues, increased hepatic glucose production, increased lipolysis in adipose tissue, and altered insulin secretion. Studies of individuals with insulin resistance, both with established T2D and high-risk individuals, have consistently demonstrated a diverse array of defects in mitochondrial function (i...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28551798/mitochondrial-proton-leak-plays-a-critical-role-in-pathogenesis-of-cardiovascular-diseases
#7
Jiali Cheng, Gayani Nanayakkara, Ying Shao, Ramon Cueto, Luqiao Wang, William Y Yang, Ye Tian, Hong Wang, Xiaofeng Yang
Mitochondrial proton leak is the principal mechanism that incompletely couples substrate oxygen to ATP generation. This chapter briefly addresses the recent progress made in understanding the role of proton leak in the pathogenesis of cardiovascular diseases. Majority of the proton conductance is mediated by uncoupling proteins (UCPs) located in the mitochondrial inner membrane. It is evident that the proton leak and reactive oxygen species (ROS) generated from electron transport chain (ETC) in mitochondria are linked to each other...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28551795/mitochondrial-ubiquitin-ligase-in-cardiovascular-disorders
#8
Tao Yu, Yinfeng Zhang, Pei-Feng Li
Mitochondrial dynamics play a critical role in cellular responses and physiological process. However, their dysregulation leads to a functional degradation, which results in a diverse array of common disorders, including cardiovascular disease. In this background, the mitochondrial ubiquitin ligase has been attracting substantial research interest in recent years. Mitochondrial ubiquitin ligase is localized in the mitochondrial outer membrane, where it plays an essential role in the regulation of mitochondrial dynamics and apoptosis...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28551791/mitochondrial-mechanosensor-microdomains-in-cardiovascular-disorders
#9
Michele Miragoli, Aderville Cabassi
The cardiomyocytes populating the 'working myocardium' are highly organized and such organization ranges from macroscale (e.g. the geometrical rod shape) to microscale (dyad/t-tubules) domains. This meticulous level of organization is imperative for assuring the normal and physiological pump-function of the heart. In the pathological cardiac tissue, the domains-related architecture is partially lost, resulting in morphological, electrical and metabolic remodeling and promoting cardiovascular diseases including heart failure and arrhythmias...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28551790/connexin-43-and-mitochondria-in-cardiovascular-health-and-disease
#10
Kerstin Boengler, Rainer Schulz
Connexin 43 (Cx43) is the major connexin protein in ventricular cardiomyocytes. Six Cx43 proteins assemble into so-called hemichannels at the sarcolemma and opposing hemichannels form gap junctions, which allow the passage of small molecules and electrical current flow between adjacent cells. Apart from its localization at the plasma membrane, Cx43 is also present in cardiomyocyte mitochondria, where it is important for mitochondrial function in terms of oxygen consumption and potassium fluxes. The expression of gap junctional and mitochondrial Cx43 is altered under several pathophysiological conditions among them are hypertension, hypertrophy, hypercholesterolemia, ischemia/reperfusion injury, post-infarction remodeling, and heart failure...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28551785/mitochondria-in-ischemic-heart-disease
#11
L Maximilian Buja
A core feature of ischemic heart disease is injury to cardiomyocytes (CMC). Ischemic CMC manifest the molecular mechanisms to undergo the major forms of cell injury and death, namely, oncotic necrosis, necroptosis, apoptosis and unregulated autophagy. Important modulators of ischemic injury are reperfusion and conditioning. Mitochondria have a major role in mediating the injury to CMC through membrane protein complexes referred to as death channels. Apoptosis is mediated by activation of a channel regulated by the Bcl-2 protein family leading to mitochondrial outer membrane permeabilization (MOMP)...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28551781/the-in-vivo-biology-of-the-mitochondrial-calcium-uniporter
#12
Julia C Liu, Randi J Parks, Jie Liu, Justin Stares, Ilsa I Rovira, Elizabeth Murphy, Toren Finkel
The identification of the molecular composition of the mitochondrial calcium uniporter has allowed for the genetic manipulation of its components and the creation of various in vivo genetic models. Here, we review the initial attempts to modulate the expression of components of the calcium uniporter in a range of organisms from plants to mammals. This analysis has confirmed the strict requirement for the uniporter for in vivo mitochondrial calcium uptake and for maintaining mitochondrial calcium homeostasis...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28551780/mitochondrial-calcium-handling-in-physiology-and-disease
#13
Veronica Granatiero, Diego De Stefani, Rosario Rizzuto
Calcium (Ca(2+)) accumulation inside mitochondria represents a pleiotropic signal controlling a wide range of cellular functions, including key metabolic pathways and life/death decisions. This phenomenon has been first described in the 1960s, but the identity of the molecules controlling this process remained a mystery until just few years ago, when both mitochondrial Ca(2+) uptake and release systems were genetically dissected. This finally opened the possibility to develop genetic models to directly test the contribution of mitochondrial Ca(2+) homeostasis to cellular functions...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28551013/citrus-nomilin-down-regulates-tnf-%C3%AE-induced-proliferation-of-aortic-smooth-muscle-cells-via-apoptosis-and-inhibition-of-i%C3%AE%C2%BAb
#14
Jinhee Kim, Sanjukta Chakraborty, G K Jayaprakasha, Mariappan Muthuchamy, Bhimanagouda S Patil
Nomilin is a bitter compound present in citrus and has been demonstrated as useful for various disease preventions through anti-proliferative, anti-inflammatory, and pro-apoptotic activities. Although in vitro disease models have shown that certain limonoids in the p38 mitogen-activated protein kinase signal cascade, the downstream signaling pathways remain unclear. In this study, the effects of nomilin on the proliferation and apoptotic pathways of human aortic smooth muscle cells (HASMCs) that forms the basis of progression of atherosclerotic diseases and restenosis was tested for the first time...
May 24, 2017: European Journal of Pharmacology
https://www.readbyqxmd.com/read/28550474/peroxisome-proliferator-activated-receptor-%C3%AE-coactivator-1%C3%AE-pgc-1%C3%AE-gene-expression-in-chronic-kidney-disease-patients-on-hemodialysis-relation-to-hemodialysis-related-cardiovascular-morbidity-and-mortality
#15
Eman Tayae Elsayed, Rasha Adel Nassra, Yasmine Salah Naga
PURPOSE: The aim of the current study was to investigate some of the key regulators of mitochondrial oxidative metabolism in ESRD patients on hemodialysis (ESRD/HD) focusing on peroxisome proliferator-activated receptor-γ-coactivator 1α (PGC-1α) gene expression and its relation to ESRD/HD-related cardiovascular diseases (CVD) and mortality in an effort to identify new potential targets for pharmacological interventions. SUBJECTS AND METHODS: The expression of PGC-1α and one of its downstream genes: COX6C were evaluated in 49 ESRD/HD patients and in 33 age- and sex-matched healthy subjects as controls using quantitative real-time PCR...
May 26, 2017: International Urology and Nephrology
https://www.readbyqxmd.com/read/28550086/the-iron-dependent-mitochondrial-superoxide-dismutase-soda-promotes-leishmania-virulence
#16
Bidyottam Mittra, Maria Fernanda Laranjeira-Silva, Danilo Ciccone Miguel, Juliana Perrone Bezerra de Menezes, Norma W Andrews
Leishmaniasis is one of the leading global neglected diseases, affecting millions of people worldwide. Leishmania infection depends on the ability of insect-transmitted metacyclic promastigotes to invade mammalian hosts, differentiate into amastigotes and replicate inside macrophages. To counter the hostile oxidative environment inside macrophages, these protozoans contain anti-oxidant systems that include iron-dependent superoxide dismutases (SODs) in mitochondria and glycosomes. Increasing evidence suggests that in addition to this protective role, Leishmania mitochondrial SOD may also initiate H2O2-mediated redox signaling that regulates gene expression and metabolic changes associated with differentiation into virulent forms...
May 26, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28549947/family-history-and-tomm40-523-interactive-associations-with-memory-in-middle-aged-and-alzheimer-s-disease-cohorts
#17
Auriel A Willette, Joseph L Webb, Michael W Lutz, Barbara B Bendlin, Alexandra M Wennberg, Jennifer M Oh, Allen Roses, Rebecca L Koscik, Bruce P Hermann, N Maritza Dowling, Sanjay Asthana, Sterling C Johnson
INTRODUCTION: Family history (FH) of Alzheimer's disease (AD) affects mitochondrial function and may modulate effects of translocase of the outer mitochondrial membrane 40 kDa (TOMM40) rs10524523 ('523) poly-T length on memory decline. METHODS: For 912 nonapolipoprotein ε4 middle-aged adults and 365 aged adults across the AD spectrum, linear mixed models gauged FH and TOMM40 '523 interactions on memory and global cognition between baseline and up to 10 years later...
May 10, 2017: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/28549842/mitochondrial-dysfunction-and-oxidative-stress-in-corneal-disease
#18
Neeru A Vallabh, Vito Romano, Colin E Willoughby
The cornea is the anterior transparent surface and the main refracting structure of the eye. Mitochondrial dysfunction and oxidative stress are implicated in the pathogenesis of inherited (e.g. Kearns Sayre Syndrome) and acquired corneal diseases (e.g. keratoconus and Fuchs endothelial corneal dystrophy). Both antioxidants and reactive oxygen species are found in the healthy cornea. There is increasing evidence of imbalance in the oxidative balance and mitochondrial function in the cornea in disease states...
May 23, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28549617/hematopathological-alterations-of-major-tumor-suppressor-cascade-vital-cell-cycle-inhibitors-and-hematopoietic-niche-components-in-experimental-myelodysplasia
#19
Ritam Chatterjee, Shubhangi Gupta, Sujata Law
Myelodysplastic syndrome (MDS) is a poorly understood dreadful hematopoietic disorder that involves maturational defect and abnormalities in blood cell production leading to dysplastic changes and peripheral blood pancytopenia. The present work aims in establishing the mechanistic relationship of the expressional alterations of major tumor suppressor cascade, vital cell cycle inhibitors and hematopoietic microenvironmental components with the disease pathophysiologies. The study involves the development of N-N' Ethylnitrosourea (ENU) induced mouse model of MDS, characterization of the disease with blood film and bone marrow smear studies, scanning electron microscopic observation, mitochondrial membrane potential determination, flowcytometric analysis of osteoblastic and vascular niche components along with the expressional study of cleaved caspase-3, PCNA, Chk-2, p53, Ndn, Gfi-1, Tie-2, Sdf-1, Gsk-3β, p18 and Myt-1 in the bone marrow compartment...
May 23, 2017: Chemico-biological Interactions
https://www.readbyqxmd.com/read/28549128/atad3-gene-cluster-deletions-cause-cerebellar-dysfunction-associated-with-altered-mitochondrial-dna-and-cholesterol-metabolism
#20
Radha Desai, Ann E Frazier, Romina Durigon, Harshil Patel, Aleck W Jones, Ilaria Dalla Rosa, Nicole J Lake, Alison G Compton, Hayley S Mountford, Elena J Tucker, Alice L R Mitchell, Deborah Jackson, Abdul Sesay, Miriam Di Re, Lambert P van den Heuvel, Derek Burke, David Francis, Sebastian Lunke, George McGillivray, Simone Mandelstam, Fanny Mochel, Boris Keren, Claude Jardel, Anne M Turner, P Ian Andrews, Jan Smeitink, Johannes N Spelbrink, Simon J Heales, Masakazu Kohda, Akira Ohtake, Kei Murayama, Yasushi Okazaki, Anne Lombès, Ian J Holt, David R Thorburn, Antonella Spinazzola
Although mitochondrial disorders are clinically heterogeneous, they frequently involve the central nervous system and are among the most common neurogenetic disorders. Identifying the causal genes has benefited enormously from advances in high-throughput sequencing technologies; however, once the defect is known, researchers face the challenge of deciphering the underlying disease mechanism. Here we characterize large biallelic deletions in the region encoding the ATAD3C, ATAD3B and ATAD3A genes. Although high homology complicates genomic analysis of the ATAD3 defects, they can be identified by targeted analysis of standard single nucleotide polymorphism array and whole exome sequencing data...
June 1, 2017: Brain: a Journal of Neurology
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