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: Orrenius Название: Mitochondrial Signaling in Health and Disease ISBN: 1439880026 ISBN-13(EAN): 9781439880029 Издательство: Taylor&Francis Рейтинг: Цена: 29093.00 р. Наличие на складе: Есть у поставщика Поставка под заказ.
Описание:
Mitochondria 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 syndrome, and cardiovascular disease. Mitochondrial Signaling in Health and Disease examines themes essential for the maintenance of the mitochondrial redox (reduction-oxidation) energy axis.
With contributions from an impressive cadre of internationally recognized scientists, the book discusses coordinated mitochondrial functions that regulate cell function by discrete signaling pathways. Topics discussed include:
Electron transport and energy production
Mitochondrial biogenesis and dynamics
Mitochondrial signaling
Apoptosis and autophagy
Pharmacology signaling
Epigenetic signaling: mitochondrial methylation and acetylation reactions
An essential resource for life and health scientists as well as pharmaceutical industry professionals, this volume highlights the importance of mitochondrial signaling and its role in establishing a harmonized communication between several cellular compartments. The information in this volume is critical to those developing mitochondrion-targeted therapies aimed at assuaging mitochondrial dysfunction or the specific factors contributing to its dysfunction.
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: Navas Название: Mitochondrial Diseases ISBN: 3030701492 ISBN-13(EAN): 9783030701499 Издательство: Springer Рейтинг: Цена: 19564.00 р. Наличие на складе: Есть у поставщика Поставка под заказ.
Описание: Mitochondrial 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.
Автор: Hongzhi Sun; Xiangdong Wang Название: Mitochondrial DNA and Diseases ISBN: 9811349290 ISBN-13(EAN): 9789811349294 Издательство: Springer Рейтинг: Цена: 20962.00 р. Наличие на складе: Поставка под заказ.
Описание: The book describes molecular principles and mechanisms by which mitochondrial DNA (mtDNA) can drive the occurrence of diseases and the latest understanding of mtDNA biology. The book explores roles of mtDNA mutation and genetic changes in cancer, with a special focus on lung cancer, and the significance of approach, application, and bioethics of mtDNA sequencing. Authors made a great effort to overview roles of mtDNA signaling pathways, base excision repair, methylation, USP30-mediated regulation, mitochondrial ribosome, autophagy pathways, or ROS-dependent signaling in the pathogenesis, diagnosis, prevention and treatment of diseases. It also demonstrates the importance of basic mitochondrial genetics and the relationship between mutations and disease phenotypes and ageing. This book covers not only the basic information of mtDNA, the relationship of mtDNA and disease, but also mtDNA in stem cell and mitochondria and metabolism etc. The book is written for biological and clinical students and researchers in the field of mtDNA–associated diseases.
Автор: Keshav K. Singh Название: Mitochondrial DNA Mutations in Aging, Disease and Cancer ISBN: 3662125110 ISBN-13(EAN): 9783662125113 Издательство: Springer Рейтинг: Цена: 16979.00 р. Наличие на складе: Есть у поставщика Поставка под заказ.
Описание: Many human genetic diseases associated with blood, brain, colon, ear, eye, heart, kidney, liver, muscle, and pancreas are caused by mutations in mitochondrial DNA. Mitochondrial DNA mutations have now been associated with aging as well as age-related degenerative diseases such as Parkinson`s, Alzheimer`s, and Huntington`s diseases.
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
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.
Автор: Oliveira, Marcos Roberto Название: Mitochondrial Dysfunction And Nanotherapeutics ISBN: 0323856667 ISBN-13(EAN): 9780323856669 Издательство: Elsevier Science Рейтинг: Цена: 22738.00 р. Наличие на складе: Поставка под заказ.
Описание: This interdisciplinary group of scholars-anthropologists, archaeologists, architects, educators, lawyers, heritage administrators, policy analysts, and consultants-make the first attempt to define and assess heritage values on a local, national and global level. Chapters range from the theoretical to policy frameworks to case studies of heritage practice, written by scholars from eight countries.
Автор: Allison, William S. Название: Mitochondrial Function, Part B,457 ISBN: 0123746221 ISBN-13(EAN): 9780123746221 Издательство: Elsevier Science Рейтинг: Цена: 26275.00 р. Наличие на складе: Поставка под заказ.
Описание: Presents methods that have been developed to assess the number and function of nuclear-encoded proteins in the mitochndrion. This work focuses on regulation of mitochondrial function and mitochondrial diseases. It includes a section emphasizing the mitochondrial defects associated with type-2 diabetes.
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.
Автор: Angelo Azzi; Katarzyna A. Nalecz; Maciej J. Nalecz Название: Anion Carriers of Mitochondrial Membranes ISBN: 3642745415 ISBN-13(EAN): 9783642745416 Издательство: Springer Рейтинг: Цена: 15372.00 р. Наличие на складе: Есть у поставщика Поставка под заказ.
Описание: All relevant structural and functional aspects of the Anion Carriers of Mitochondrial Membranes as well as isolation procedures, reconstitution experiments and biogenesis of the carriers, kinetics of transport as well as the metabolic regulation mechanisms are treated in this volume.
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