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Mitochondrial Biology and Experimental Therapeutics
by Paulo J. OliveiraThis book addresses the therapeutic strategies to target mitochondrial metabolism in diseases where the function of that organelle is compromised, and it discusses the effective strategies used to create mitochondrial-targeted agents that can become commercially available drug delivery platforms. The consistent growth of research focused in understanding the multifaceted role of mitochondria in cellular metabolism, controlling pathways related with cell death, and ionic/redox regulation has extended the research of mitochondrial chemical-biological interactions to include various pharmacological and toxicological applications. Not only does the book extensively cover basic mitochondrial physiology, but it also links the molecular interactions within these pathways to a variety of diseases. It is one of the first books to combine state-of-the-art reviews regarding basic mitochondrial biology, the role of mitochondrial alterations in different diseases, and the importance of that organelle as a target for pharmacological and non-pharmacological interventions to improve human health. The different chapters highlight the chemical-biological linkages of the mitochondria in context with drug development and clinical applications.
Mitochondrial DNA
by Jeffrey A StuartSince the publication of the first edition, the number of unique heritable mtDNA mutations recognized as being associated with bioenergetic dysfunction, cell death and disease has grown. Likewise, our understanding of the basic biology of somatic mtDNA mutations continues to improve. In Mitochondrial DNA: Methods and Protocols, Second Edition, specialists from eight countries share their expertise, providing detailed protocols for studying many aspects of mtDNA. The volume is divided into three sections, which cover the transduction of information from mtDNA to functionally active respiratory complexes, mitochondrial reactive oxygen species (ROS) production as well as mtDNA damage and its repair, and the identification and quantification of heteroplasmic mtDNA mutations. Written in the highly successful Methods in Molecular BiologyTM series format, chapters include introductions to their respective topics, lists of the necessary materials and reagents, step-by-step, readily reproducible laboratory protocols, and notes sections, highlighting tips on troubleshooting and avoiding known pitfalls. Comprehensive and authoritative, Mitrochondrial DNA: Methods and Protocols, Second Edition promises to aid researchers in further expanding our knowledge of this vital area of cell biology.
Mitochondrial DNA
by Matthew MckenzieThis third edition is comprised of well-established protocols that are considered the gold standard in the field as well as new methodologies for mitochondrial DNA analysis. of Mitochondrial DNA: Methods and Protocols describes protocols for detecting mutations in mitochondrial DNA, techniques to assess mitochondrial DNA damage, visualization of mitochondrial DNA in situ, detection of mitochondrial DNA nucleoids within the mitochondria, methods for analyzing mitochondrial DNA replication, mitochondrial DNA-encoded protein translation and mitochondrial DNA copy number, the latest technologies for modifying the mitochondrial genome and methods for the purification of proteins involved in the replication and transcription of mitochondrial DNA. Written for the Methods in Molecular Biology series, chapters include introductions to their respective topics, lists of the necessary materials and reagents, step-by-step, readily reproducible laboratory protocols and tips on troubleshooting and avoiding known pitfalls. Authoritative and practical, Mitochondrial DNA: Methods and Protocols, Third Edition will be useful not only for mitochondrial researchers but also for scientists studying human diseases where mitochondrial DNA variation has been recognized as an important pathogenic factor, including cancer and neurodegeneration.
Mitochondrial DNA
by William C. CopelandInternationally recognized authorities describe in great detail the methods they have perfected to analyze mtDNA and the proteins involved in its maintenance. The analytical techniques cover the purification of mtDNA from a variety of sources and the analysis of DNA for both deletions, point mutations, and damage, for replication intermediates, and for following the fate of mtDNA outside of the mitochondria. Additional analytical methods are presented for analyzing the proteins and enzymes that maintain mtDNA. Comprehensive and timely, Mitochondrial DNA: Methods and Protocols offers both basic and clinical researchers proven cutting-edge methods for analyzing mtDNA and illuminating the role it plays in the aging process, apoptosis, and possibly some cancers.
Mitochondrial DNA, Mitochondria, Disease and Stem Cells
by Justin C. St. JohnThis volume investigates how the mitochondrial genome is transmitted, segregated, and inherited. It starts by describing mtDNA mutations and deletions and how these impact on the offspring's well-being. It progresses to discuss how mutations to the mtDNA-nuclear-encoded transcription, replication and translational factors lead to mtDNA-depletion syndromes and how these affect cellular function and lead to the pathology of human mitochondrial disease. It also highlights the importance of the mitochondrial assembly factors and how mutations to these can lead to mitochondrial disease. The reader is then introduced to how mtDNA is transmitted through the oocyte and how stem cells can be used to study mitochondrial biogenesis and mtDNA replication and transcription in undifferentiated pluripotent and differentiating cells and how mitochondria adapt during this process. It then discusses how diseases like cancer are initiated and regulated by mutations to mitochondrial DNA and dysfunctional mitochondria. Finally, it draws on assisted reproductive technologies to discuss how some of these approaches might be adapted to prevent the transmission of mutant and deleted mtDNA from one generation to the next.
Mitochondrial DNA: Methods and Protocols (Methods in Molecular Biology #2615)
by Thomas J. Nicholls Jay P. Uhler Maria FalkenbergThis volume compiles a comprehensive range of methods to study key aspects of mitochondrial DNA including nucleoid structure and packaging, replication, genome integrity, and disease. Chapters are organized into eight methodological sections that cover in vitro and in vivo methods, including for mtDNA isolation, visualization, deep sequencing, gene editing, and diagnostic aspects of mtDNA disease. Written in the format of the highly successful Methods in Molecular Biology series, each chapter includes an introduction to the topic, lists necessary materials and methods, includes tips on troubleshooting and known pitfalls, and step-by-step, readily reproducible protocols. Authoritative and cutting-edge, Mitochondrial DNA: Methods and Protocols aims to be useful and informative for researchers and clinicians with an interest in mitochondrial DNA.
Mitochondrial Diseases (Life Science Research Fundamentals)
by WileyThis collection of reviews and protocols provides the reader with an introduction to the current state of knowledge on how various diseases are related to mitochondrial dysfunction. Mitochondria contain their own genome, a small, circular double-stranded DNA (mtDNA), and alterations in mtDNA may play an important role in the multistep carcinogenesis of at least some types of human cancer. In addition to mutations of mtDNA, many mitochondrial syndromes are due to abnormalities in nuclear genes related to oxidative phosphorylation (OXPHOS). Mitochondrial tRNA (MTT) gene mutations are an important cause of human morbidity and are associated with a wide range of pathology, from isolated organ?]specific diseases such as myopathy or hearing loss, to multisystem disorders with encephalopathy, gastrointestinal dysmotility, and life?]threatening cardiomyopathy. The relationship of the mitochondrion organelle to aging and longevity is also discussed. Laboratory protocols describe methodology to characterize mtDNA heteroplasmy by parallel sequencing. Each eukaryotic cell contains hundreds of mitochondria with hundreds of mitochondria genomes. Mutant and wild-type mtDNA may co-exist as heteroplasmy, and cause human disease. The purpose of this protocol is to simultaneously determine mtDNA sequence and quantify the heteroplasmic level. Another protocol describes procedures for obtaining tissue sections and cell material suitable for histological evaluation of OXPHOS activity and integrity and immunodetection of the complexes in tissue from patients suspected of mitochondrial disease. Emphasis lies on the diagnostic potential of these techniques to differentiate mtDNA from nuclear mutations. This e-book — a curated collection from eLS, WIREs, and Current Protocols — offers a fantastic introduction to the field of mitochondrial diseases for students or interdisciplinary collaborators.
Mitochondrial Diseases: Theory, Diagnosis and Therapy
by Plácido Navas Leonardo SalviatiMitochondrial diseases comprise a clinically and genetically heterogeneous group of rare disorders that may affect virtually any system of the body at any age. Due to their complexity, understanding and diagnosing these diseases requires a multidisciplinary approach.This book provides an update on the major features of human mitochondrial diseases: genetic bases, pathophysiology, diagnosis, and treatment, and of the new technologies involved in the diagnosis and on the characterization of patients. The 11 chapters examine the unique complex interactions between the mitochondrial and the nuclear genomes involved in the biogenesis and the regulation of the mitochondrial respiratory chain, and their relevance to human disease. We discuss the traditional biochemical and genetic approaches, as well as the new omic technologies, and the cellular and animal models used in mitochondrial research. The last chapter is dedicated to the current treatment options.Authors are worldwide experts in these fields and integrate expertise in both basic science and clinical research.This book is particularly important for both scientists and clinicians interested in the diagnosis and treatment of these diseases.
Mitochondrial Disorders
by Lee-Jun C. WongDue to the complexity of the mitochondrial system and the myriad genes involved in their function, the clinical manifestations of mitochondrial diseases are remarkably variable and heterogeneous. The laboratory and clinical methodologies used to evaluate dysfunction may vary widely, and thusly a systematic presentation of the numerous protocols that are applied to the assessment of these clinically and genetically heterogeneous disorders has proven to be essential. Mitochondrial Disorders: Biochemical and Molecular Analysis strives to fill this need with a collection of key protocols provided by leading experts in the field. Beginning with overviews of complexity of mitochondrial and nuclear genome disorders, the book continues with a section devoted to current biochemical protocols and a part focused on the DNA-based approaches used to identify molecular defects. Written in the highly successful Methods in Molecular BiologyTM series format, chapters contain introductions to their respective topics, lists of the necessary materials and reagents, step-by-step, readily reproducible laboratory protocols, and tips on troubleshooting and avoiding known pitfalls. Detailed and authoritative, Mitochondrial Disorders: Biochemical and Molecular Analysis serves as a vital guide to researchers seeking to understand this incredibly complicated type of breakdown in cellular biology.
Mitochondrial Disorders Caused by Nuclear Genes
by Lee-Jun C. WongMitochondrial cytopathies are mutations in the inherited maternal mitochondrial genome, or the nuclear DNA-mutation. Mitochondrial respiratory chain disorders (RCD) are a group of genetically and clinically heterogeneous diseases, due to the fact that protein components of the respiratory chain are encoded by both mitochondrial and nuclear genomes and are essential in all cells. In addition, the biogenesis, structure and function of mitochondria, including DNA replication, transcription, and translation, all require nuclear encoded genes. Since mitochondria are present in every cell, every tissue, mitochondrial disorder usually affects multiple organs.
Mitochondrial Dynamics and Neurodegeneration
by Bingwei LuMitochondria are essential organelles in eukaryotic cells that control such diverse processes as energy metabolism, calcium buffering, and cell death. Recent studies have revealed that changes in mitochondrial morphology by fission and fusion, a process known as mitochondrial dynamics, is particularly important for neuronal function and survival. Defects in this process are commonly found in neurodegenerative diseases, offering a new paradigm for investigating mechanisms of neurodegeneration. To provide researchers working on neurodegenerative diseases and mitochondria with updated information on this rapidly progressing field, we have invited experts in the field to critically review recent progresses and identify future research directions. The topics include genetics of mitochondrial dynamics, mitochondrial dynamics and bioenergetics, autophagy, apoptosis, and axonal transport, and its role in neurological diseases, including Alzheimer's, Parkinson's, and Huntington's diseases.
Mitochondrial Dysfunction Caused by Drugs and Environmental Toxicants
by Yvonne Will James A. DykensDeveloped as a one-stop reference source for drug safety and toxicology professionals, this book explains why mitochondrial failure is a crucial step in drug toxicity and how it can be avoided. • Covers both basic science and applied technology / methods• Allows readers to understand the basis of mitochondrial function, the preclinical assessments used, and what they reveal about drug effects• Contains both in vitro and in vivo methods for analysis, including practical screening approaches for drug discovery and development• Adds coverage about mitochondrial toxicity underlying organ injury, clinical reports on drug classes, and discussion of environmental toxicants affecting mitochondria
Mitochondrial Dysfunction in Neurodegenerative Disorders
by Michael R. Duchen Amy K. Reeve Eve M. Simcox Doug M. TurnbullThis second edition brings together up-to-date contributionsfrom leaders in the field internationally on the various ways in whichmitochondrial dysfunction contributes to the pathogenesis of neurodegenerativediseases, including Parkinson's disease, Alzheimer's disease and multiplesclerosis. The reader is guided through the basic functions of mitochondria andthe mechanisms that lead to their dysfunction, and on to the consequences ofthis dysfunction for neuronal function before finishing with the modelling ofthese disorders and discussion of new potential therapeutic targets. Additional chapters have been added to the book to reflectadvances in the field and there are many new contributors and topics, includinghow mitochondria are degraded and the interaction of the mitochondria withpathologically relevant proteins. Mitochondrial Dysfunction in Neurodegenerative Disordersprovides an accessible, authoritative guide to this important area forneurologists; research and clinical neuroscientists; neuropathologists; andresidents with an interest in clinical research.
Mitochondrial Dysfunction in Neurodegenerative Disorders
by Kim Jennifer Krishnan Doug M Turnbull Amy Katherine Reeve Michael R. DuchenAs age related diseases increase in prevalence and impact more significantly on medical resources it is imperative to understand these diseases and the mechanisms behind their progression. New research has stimulated a growing interest in mitochondrial involvement in neurodegenerative disorders such as Parkinson's disease, Alzheimer's disease and multiple sclerosis and the mechanisms which lead from mitochondrial dysfunction to neurodegeneration. Mitochondrial Dysfunction in Neurodegenerative Disorders brings together contributions from leaders in the field internationally on the various ways in which mitochondrial dysfunction contributes to the pathogenesis of these diseases, guiding the reader through the basic functions of mitochondria and the mechanisms that lead to their dysfunction, to the consequences of this dysfunction on neuronal function before finishing with the modelling of these disorders and discussion of new potential therapeutic targets. Mitochondrial Dysfunction in Neurodegenerative Disorders provides an accessible, authoritative guide to this important area for neurologists; research and clinical neuroscientists; neuropathologists; and residents with an interest in clinical research.
Mitochondrial Function In Vivo Evaluated by NADH Fluorescence
by Avraham MayevskyThis book covers both the technological development and biomedical applications of NADH fluorescence. Topics covered include perspectives on the history of monitoring NADH fluorescence, the relationship between mitochondrial function and other functions at the tissue level, responses of NADH to physiological and pathophysiological conditions, monitoring of NADH in the human brain and other organs, and metabolism. It also includes an in-depth look at flavoprotein (Fp) fluorescence and NADH in relation to redox state. This is an ideal book for biomedical engineers, researchers, and graduate students interested in learning the biomedical applications of NADH fluorescence. This book also: Covers multisite monitoring of NADH, as well as multiparametric responses of NADH to physiological and pathophysiological conditions, and monitoring of various organs in various animal models Describes the relationship between brain activation (i. e. epileptic activity and cortical spreading depression) and NADH redox state Presents the effects of hypoxia,hyperbaric hyperoxia, and ischemia on brain NADH fluorescence and other tissue physiological parameters About the Author Avraham Mayevsky, Ph. D. is a Professor Emeritus in theFaculty of Life Sciences and the Brain Research Center at Bar Ilan University, Israel. He has published more than two hundred papers in the field of mitochondrial function and tissue physiology in vivo under pathophysiological conditions.
Mitochondrial Function in Lung Health and Disease
by Narasimham L. Parinandi Viswanathan NatarajanMitochondria, often referred to as the "powerhouses" of the cell, generate adenosine triphosphate (ATP) by oxidative phosphorylation or OXPHOS, and maintain cellular homeostasis. In addition to generating ATP, mitochondria are involved in regulation of cell cycle, proliferation, free radical production, innate immune responses and apoptosis. Mitochondrial Function in Lung Health and Disease fills the current gap in the literature and outlines the growing clinical relevance of mitochondrial dysfunction Currently, there is no overview on the role of mitochondria in pulmonary diseases and this volume focuses on the mitochondrial metabolism, redox signaling, and mechanisms of mitochondrial pathways in lung injury, inflammation, repair and remodeling Furthermore, in addition to their well-recognized role in cellular energy production and apoptosis, mitochondria appear to play a role in many respiratory diseases and lung cancer. Chapters are written by top notch researchers and clinicians and outline the evidence for mitochondrial biogenesis in inhalational lung injury, COPD and asthma.
Mitochondrial Genetics and Cancer
by Gabriel D. DakuboThe pivotal role of mitochondrial functions in carcinogenesis is quite well established. However, the critical role of mitochondrial genome alterations to cancer development is muted in traditional textbooks. Recent scientific efforts have provided unequivocal evidence for mitochondrial genome mutations and content changes in cancer development, progression, and therapy. This pioneering book is a unique compilation of mitochondrial genome alterations in cancer. While primarily focused on the emerging role of mitochondrial genome changes, bioenergetics and signaling pathways, attention is also given to the metabolic transformations of the cancer cell, as well as the established altered cell death processes that underlie cancer evolution and treatment resistance.
Mitochondrial Mechanisms of Degeneration and Repair in Parkinson's Disease
by Lori M. BuhlmanThe first volume to bring together various theories of how aberrations in mitochondrial function and morphology contribute to neurodegeneration in idiopathic and familial forms of Parkinson's disease, to comprehensively review the current search for therapies, and to propose molecules involved in specific functions as attractive therapeutic targets. It is expected to facilitate critical thought and discussion about the fundamental aspects of neurodegeneration in Parkinson's disease and foster the development of therapeutic strategies among biomedical sciences researchers and graduate students. Theories of idiopathic Parkinson's etiology support roles for chronic inflammation and exposure to heavy metals or pesticides. Interestingly, as this project proposes, a case can be made that abnormalities in mitochondrial morphology and function are at the core of each of these theories. In fact, the most common approach to the generation of animal and cell-culture models of idiopathic Parkinson's disease involves exposure to mitochondrial toxins. Even more compelling is the fact that most familial patients harbor genetic mutations that cause disruptions in normal mitochondrial morphology and function. While there remains to be no effective treatment for Parkinson's disease, efforts to postpone onset, prevent and "cure" mitochondrial aberrations and neurodegeneration associated with Parkinson's disease in various models are encouraging. While only about ten percent of Parkinson's patients inherit disease-causing mutations, discovering common mechanisms by which familial forms of Parkinson's disease manifest will likely shed light on the pathophysiology of the more common idiopathic form and provide insight to the general process of neurodegeneration, thus revealing therapeutic targets that will become more and more accessible as technology improves.
Mitochondrial Medicine
by Volkmar Weissig Marvin EdeasThis expert volume covers an interdisciplinary and rapidly growing area of biomedical research comprising genetic, biochemical, pathological, and clinical studies aimed at the diagnosis and therapy of human diseases which are either caused by or associated with mitochondrial dysfunction. It dedicates itself to showcasing the tremendous efforts and the progress that has been made over the last decades in developing techniques and protocols for probing, imaging, and manipulating mitochondrial functions. Mitochondrial Medicine: Volume I, Probing Mitochondrial Function focuses on methods being used for the assessment of mitochondrial function under physiological conditions as well as in healthy isolated mitochondria. Written in the highly successful Methods in Molecular Biology series format, chapters include introductions to their respective topics, lists of the necessary materials and reagents, step-by-step, readily reproducible laboratory protocols, and tips on troubleshooting and avoiding known pitfalls. Comprehensive and practical, Mitochondrial Medicine provides an essential source of know-how and inspiration to all researchers who are fascinated by this tiny organelle that seems so clearly to control the life and death of a single cell and whole organisms alike.
Mitochondrial Medicine: Volume 1: Targeting Mitochondria (Methods in Molecular Biology #2275)
by Volkmar Weissig Marvin EdeasThis second edition offers 88 chapters divided among three volumes providing the most comprehensive source of know-how in the wide-ranging field of Mitochondrial Medicine. Volume I guides readers through chapters on QSAR models, DQAsomes, synthesis of Triphenylphosphonium Phospholipid Conjugates, testing of novel isomeric mitochondriotropic derivatives, mTRIP, mitochondria-targeted imaging nanoplatforms, live-cell assessment, mitochondrial coenzyme Q10, rat liver Mitochondrial Lipidome, and Mito-SinCe2. Written in the highly successful Methods in Molecular Biology series format, chapters include introductions to their respective topics, application details for both the expert and non-expert reader, and tips on troubleshooting and avoiding known pitfalls. Authoritative and accessible, Mitochondrial Medicine, Second Edition, Volume 1: Targeting Mitochondria aims to be a comprehensive source of know-how in the wide-ranging field of Mitochondrial Medicine.
Mitochondrial Oxidative Phosphorylation
by Bernhard KadenbachThis book will describe the nuclear encoded genes and their expressed proteins of mitochondrial oxidative phosphorylation. Most of these genes occur in eukaryotic cells, but not in bacteria or archaea. The main function of mitochondria, the synthesis of ATP, is performed at subunits of proton pumps (complexes I, III, IV and V), which are encoded on mitochondrial DNA. The nuclear encoded subunits have mostly a regulatory function. However, the specific physiological functions of the nuclear encoded subunits of complexes I, III, IV, and V are mostly unknown. New data indicates that they are essential for life of higher organisms, which is characterized by an adult life without cell division (postmeiotic stage) in most tissues, after the juvenile growth. For complex IV (cytochrome c oxidase) some of these subunits occur in tissue-specific (subunits IV, VIa, VIb, VIIa, VIII), developmental-specific (subunits IV, VIa, and VIIa) as well as species-specific isoforms. Defective genes of some subunits were shown to induce mitochondrial diseases. Mitochondrial genes and human diseases will also be covered.
Mitochondrial Regulation
by Carlos M. Palmeira Anabela P. RoloIn this volume expert researchers in the field detail the many methods for further research into regulation of mitochondrial function. Chapters focus on mitochondria with other cellular components, discussing how these interactions influence the dynamics of mitochondrial structure and biogenesis. Written in the highly successful Methods in Molecular Biology series format, chapters include introductions to their respective topics, lists of the necessary materials and reagents, step-by-step, readily reproducible laboratory protocols, and key tips on troubleshooting and avoiding known pitfalls. Authoritative and practical, Mitochondrial Regulation: Methods and Protocols seeks to aid advanced undergraduates, graduates, postgraduates, and beginning researchers in the areas of molecular and cellular biology, biochemistry and bioenergetics.
Mitochondrial Regulation: Methods and Protocols (Methods in Molecular Biology #2310)
by Carlos M. Palmeira Anabela P. RoloThis fully updated edition explores the different pathways that converge into the regulation of mitochondrial function. The book integrates mitochondria with other cellular components, discussing the dynamic properties of mitochondria with an emphasis on how these processes respond to signaling events and how they affect cellular metabolism. Written for the highly successful Methods in Molecular Biology series, chapters include introductions to their respective topics, lists of the necessary materials and reagents, step-by-step, readily reproducible laboratory protocols, and tips on troubleshooting and avoiding known pitfalls. Authoritative and up-to-date, Mitochondrial Regulation: Methods and Protocols, Second Edition is an ideal guide for advanced undergraduates, graduates, postgraduates, and beginning researchers in the areas of molecular and cellular biology, biochemistry, and bioenergetics.
Mitochondrial Replacement Techniques: Ethical, Social, and Policy Considerations
by Engineering Medicine National Academies of SciencesMitochondrial replacement techniques (MRTs) are designed to prevent the transmission of mitochondrial DNA (mtDNA) diseases from mother to child. While MRTs, if effective, could satisfy a desire of women seeking to have a genetically related child without the risk of passing on mtDNA disease, the technique raises significant ethical and social issues. It would create offspring who have genetic material from two women, something never sanctioned in humans, and would create mitochondrial changes that could be heritable (in female offspring), and therefore passed on in perpetuity. The manipulation would be performed on eggs or embryos, would affect every cell of the resulting individual, and once carried out this genetic manipulation is not reversible. Mitochondrial Replacement Techniques considers the implications of manipulating mitochondrial content both in children born to women as a result of participating in these studies and in descendants of any female offspring. This study examines the ethical and social issues related to MRTs, outlines principles that would provide a framework and foundation for oversight of MRTs, and develops recommendations to inform the Food and Drug Administration’s consideration of investigational new drug applications.
Mitochondrial Signaling in Health and Disease (Oxidative Stress and Disease)
by Enrique Cadenas Lester Packer Sten OrreniusMitochondria have traditionally been associated with metabolic functions; however recent research has uncovered a central role for these organelles in cell signaling, cell survival, and cell death. Mitochondrial dysfunction is a factor in a myriad of pathophysiological conditions, including age-related neurodegenerative disorders, cancer, metabolic