Автор: Patrick Lestienne Название: Mitochondrial Diseases ISBN: 3642641660 ISBN-13(EAN): 9783642641664 Издательство: Springer Рейтинг: Цена: 15672.00 р. Наличие на складе: Есть у поставщика Поставка под заказ.
Описание: For those like me who witnessed the beginning of the adventure of human mitochon- drial pathology, one can only be astounded by the extent and unexpectedness of what the field has come to offer. Extent because nobody could have imagined the sheer size of the domain. Unexpectedness because hitherto it was impossible to imagine the clinical polymorphism that this pathology would represent. The starting point was clear. Initially, there was the exceptional, and for a long time unique, observation of euthyroidian hypermetabolism that Luft and colleagues analyzed remarkably in biochemical and clinical terms. Thereafter, there was the support provided by the electron microscopy studies of Afzelius, and the very first visualization of mitochondrial abnormalities. That was way back in 1958. A few years later, progress in the cytology and cytochemistry of skeletal muscle tissue was to provide the means of detecting such abnormalities by examining sections with light microscopy. The colorful term "ragged red fibers", coined by W. K. Engel, became uni- versally accepted, and this typical aspect with Gomori trichrome stain was to throw light on the frequency with which these mitochondrial abnormalities could occur under pathological conditions which, until then, had remained a total mystery regard- ing their mechanism: syndromes such as the ocular myopathies with their descending evolution and the oculocraniosomatic syndromes. We were at the beginning of the 1970s.
Название: Mitochondrial disorders caused by nuclear genes ISBN: 1489992413 ISBN-13(EAN): 9781489992413 Издательство: Springer Рейтинг: Цена: 26120.00 р. Наличие на складе: Есть у поставщика Поставка под заказ.
Описание: This book surveys mutations in the inherited maternal mitochondrial genome, or the nuclear DNA-mutation, covering the biochemical, molecular, clinical, and genetic aspects of syndromes and heterogeneous mitochondrial disease caused by specific genes.
Описание: This volume in the Encyclopedia of Sustainability Science and Technology, Second Edition, describes the breadth of science and engineering knowledge critical to advancing sustainable built environments, from architecture and design, mechanical engineering, lighting, and materials to water and energy, public policy, and economics.
Описание: This book describes the author’s work on the development of sequence-specific DNA binders for the therapy of mitochondrial diseases. In the first chapter, the author provides a detailed background of pyrrole–imidazole polyamides (PIPs) and mitochondrial disease research followed by chapters presenting the author's own research and discoveries. Firstly, the developed compounds called MITO-PIPs, which recognize specific sequences in mitochondrial DNA, are presented. The following chapter demonstrates how, by introducing a DNA alkylating reagent into a MITO-PIP that recognizes the adjacent sequence to a target mutation, the copy number of mutated mitochondrial DNA was successfully reduced in live cells. Furthermore, because nuclear DNA is another important target for treating mitochondrial diseases, the author demonstrated that tri-arginine vectors can enhance nuclear uptake of PIPs and improve their biological activity in cells. This work will attract readers’ interest because it paves the way for a transgene-free chemical gene therapy for mitochondrial diseases. The book includes a detailed description of experimental procedures, especially compound synthesis. This description helps readers to have a clear image of the author’s studies and to perform similar and extended studies themselves.
Автор: Navas Placido, Salviati Leonardo Название: Mitochondrial Diseases: Theory, Diagnosis and Therapy ISBN: 3030701468 ISBN-13(EAN): 9783030701468 Издательство: Springer Цена: 19564.00 р. Наличие на складе: Есть у поставщика Поставка под заказ.
Описание: PrefaceChapter 1 Two genomes connection: biogenesis of mitochondria respiratory chain Chapter 2 Nuclear DNA: mutations in structural subunits and assembly factorsChapter 3 Defects of mtDNAChapter 4 MtDNA maintenance genes and depletionChapter 5 Defects in mitochondrial protein synthesisChapter 6 Defects in mitochondria dynamicsChapter 7 CoQ biosynthesis disordersChapter 8 Cytochrome c mutationsChapter 9 Biochemical approaches for mitochondrial diseases diagnosisChapter 10 Molecular genetics in the Next Generation Sequencing era Chapter 11 Model cells and organismsChapter 12 Metabolomics and proteomics: complementary toolsChapter 13 Therapies approaches in mitochondrial diseasesIndex
Автор: Michael Forte; Marco Colombini Название: Molecular Biology of Mitochondrial Transport Systems ISBN: 3642789382 ISBN-13(EAN): 9783642789380 Издательство: Springer Рейтинг: Цена: 18167.00 р. Наличие на складе: Есть у поставщика Поставка под заказ.
Описание: Proceedings of the NATO Advanced Research Workshop on Molecular Biology of Mitochondrial Transport Systems, held at Il Ciocco, Italy, September 17 - 21, 1992
Описание: This volume brings together various theories of how aberrations in mitochondrial function and morphology contribute to neurodegeneration in idiopathic and familial forms of Parkinson’s disease. Moreover, it comprehensively reviews the current search for therapies, and proposes how molecules are 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 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, prevent and “cure” onset 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.
Автор: Strack Stefan, Usachev Yuriy M. Название: Techniques to Investigate Mitochondrial Function in Neurons ISBN: 1493983296 ISBN-13(EAN): 9781493983292 Издательство: Springer Рейтинг: Цена: 19564.00 р. Наличие на складе: Есть у поставщика Поставка под заказ.
Описание:
Neuromethods Preface... Preface... Table of Contents... Contributing Authors...
1. Three-Dimensional Reconstruction of Neuronal Mitochondria by Electron Tomography Guy Perkins 2. Measuring Mitochondrial Shape with ImageJ Ronald A. Merrill, Kyle H. Flippo, and Stefan Strack 3. Live Imaging Mitochondrial Transport in Neurons Meredith M. Course, Chung-Han Hsieh, Pei-I Tsai, Jennifer A. Codding-Bui, Atossa Shaltouki, and Xinnan Wang 4. Techniques to Investigate Bioenergetics of Mitochondria William I. Sivitz 5. Respirometry in Neurons Liang Zhang and Eugenia Trushina 6. Measuring ATP in Axons with FRET Lauren Y. Shields, Bryce A. Mendelsohn, and Ken Nakamura 7. Characterizing Metabolic States using Fluorescence Lifetime Imaging Microscopy (FLIM) of NAD(P)H Thomas S. Blacker and Michael R. Duchen 8. Techniques for Simultaneous Mitochondrial and Cytosolic Ca2+ Imaging in Neurons Jacob E. Rysted, Zhihong Lin, and Yuriy M. Usachev 9. Live Imaging of Mitochondrial ROS Production and Dynamic Redox Balance in Neurons Karolina Can, Sebastian Kьgler, and Michael Mьller 10. Monitoring of Permeability Transition Pore Openings in Isolated Individual Brain Mitochondria Nickolay Brustovetsky and Tatiana Brustovetsky 11. Examination of Mitochondrial Ion Conductance by Patch Clamp in Intact Neurons and Mitochondrial Membrane Preparations Elizabeth A. Jonas and Nelli Mnatsakanyan 12. Monitoring of Permeability Transition Pore Openings in Mitochondria of Cultured Neurons Tatiana Brustovetsky and Nickolay Brustovetsky 13. Protocols for Assessing Mitophagy in Neuronal Cell Lines and Primary Neurons Ruben K. Dagda and Monica Rice 14. Examining Mitochondrial Function at Synapses In Situ Gregory T. Macleod and Maxim V. Ivannikov 15. Proteomic Analysis of Neuronal Mitochondria Kelly L. Stauch and Howard S. Fox 16. Measuring Mitochondrial Pyruvate Oxidation Lawrence R. Gray, Alix A.J. Rouault, Lalita Oonthonpan, Adam J. Rauckhorst, Julien A. Sebag, and Eric B. Taylor
Автор: Paulo J. Oliveira Название: Mitochondrial Biology and Experimental Therapeutics ISBN: 3030103587 ISBN-13(EAN): 9783030103583 Издательство: Springer Рейтинг: Цена: 25155.00 р. Наличие на складе: Есть у поставщика Поставка под заказ.
Описание: This 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.
Автор: Carlos M. Palmeira; Ant?nio J. Moreno Название: Mitochondrial Bioenergetics ISBN: 1493958372 ISBN-13(EAN): 9781493958375 Издательство: Springer Рейтинг: Цена: 20263.00 р. Наличие на складе: Есть у поставщика Поставка под заказ.
Описание: This Methods in Molecular Biology (TM) book offers practical principles and assays designed to derive quantitative assessment of each set of parameters that reflect different aspects of mitochondrial bioenergetics. Includes protocols, materials lists and tips.
Автор: Anna Gvozdj?kov? Название: Mitochondrial Medicine ISBN: 9400786905 ISBN-13(EAN): 9789400786905 Издательство: Springer Рейтинг: Цена: 32142.00 р. Наличие на складе: Есть у поставщика Поставка под заказ.
Описание: Mitochondrial medicine deals with diseases that are related to mitochondrial dysfunction due to a number of causes from free radical damage to genetic mutation.
Автор: Justin C. St. John Название: Mitochondrial DNA, Mitochondria, Disease and Stem Cells ISBN: 1627038671 ISBN-13(EAN): 9781627038676 Издательство: Springer Рейтинг: Цена: 22201.00 р. Наличие на складе: Есть у поставщика Поставка под заказ.
Описание: 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.
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