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Mitochondrial Diseases, Navas


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Автор: Navas
Название:  Mitochondrial Diseases
ISBN: 9783030701499
Издательство: Springer
Классификация:



ISBN-10: 3030701492
Обложка/Формат: Soft cover
Страницы: 305
Вес: 0.48 кг.
Дата издания: 14.08.2022
Язык: English
Издание: 1st ed. 2021
Иллюстрации: 23 illustrations, color; 6 illustrations, black and white; vi, 305 p. 29 illus., 23 illus. in color.; 23 illustrations, color; 6 illustrations, black
Размер: 235 x 155
Читательская аудитория: Professional & vocational
Основная тема: Biomedicine
Подзаголовок: Theory, Diagnosis and Therapy
Ссылка на Издательство: Link
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Поставляется из: Германии
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.
Дополнительное описание: Chapter 1. MTOCHONRIAL NEUROLOGY: A TALE OF TWO GENOMES (Salvatore DiMauro and Emanuele Barca).- Chapter 2. Mutations in assembly factors required for the biogenesis of mitochondrial respiratory chain (Cristina Cerqua, Lisa Buson and Eva Trevisson).- Chap


Sequence-Specific DNA Binders for the Therapy of Mitochondrial Diseases

Автор: Hidaka Takuya
Название: Sequence-Specific DNA Binders for the Therapy of Mitochondrial Diseases
ISBN: 9811684359 ISBN-13(EAN): 9789811684357
Издательство: Springer
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Цена: 139750.00 T
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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.

Mitochondrial Diseases

Автор: Patrick Lestienne
Название: Mitochondrial Diseases
ISBN: 3642641660 ISBN-13(EAN): 9783642641664
Издательство: Springer
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Цена: 104480.00 T
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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.

Mitochondrial Diseases: Theory, Diagnosis and Therapy

Автор: Navas Placido, Salviati Leonardo
Название: Mitochondrial Diseases: Theory, Diagnosis and Therapy
ISBN: 3030701468 ISBN-13(EAN): 9783030701468
Издательство: Springer
Цена: 130430.00 T
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

Molecular Biology of Mitochondrial Transport Systems

Автор: Michael Forte; Marco Colombini
Название: Molecular Biology of Mitochondrial Transport Systems
ISBN: 3642789382 ISBN-13(EAN): 9783642789380
Издательство: Springer
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Цена: 121110.00 T
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Описание: Proceedings of the NATO Advanced Research Workshop on Molecular Biology of Mitochondrial Transport Systems, held at Il Ciocco, Italy, September 17 - 21, 1992

Mitochondrial disorders caused by nuclear genes

Название: Mitochondrial disorders caused by nuclear genes
ISBN: 1489992413 ISBN-13(EAN): 9781489992413
Издательство: Springer
Рейтинг:
Цена: 174130.00 T
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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.

Mitochondrial Mechanisms of Degeneration and Repair in Parkinson`s Disease

Автор: Buhlman
Название: Mitochondrial Mechanisms of Degeneration and Repair in Parkinson`s Disease
ISBN: 3319421379 ISBN-13(EAN): 9783319421377
Издательство: Springer
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Цена: 158380.00 T
Наличие на складе: Есть у поставщика Поставка под заказ.
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.

Techniques to Investigate Mitochondrial Function in Neurons

Автор: Strack Stefan, Usachev Yuriy M.
Название: Techniques to Investigate Mitochondrial Function in Neurons
ISBN: 1493983296 ISBN-13(EAN): 9781493983292
Издательство: Springer
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Цена: 130430.00 T
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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


Handbook of Mitochondrial Dysfunction

Автор: Shamim I. Ahmad
Название: Handbook of Mitochondrial Dysfunction
ISBN: 1138336084 ISBN-13(EAN): 9781138336087
Издательство: Taylor&Francis
Рейтинг:
Цена: 178640.00 T
Наличие на складе: Невозможна поставка.
Описание: Mitochondria produce the chemical energy necessary for eukaryotic cell functions; hence mitochondria are an essential component of health, playing roles in both disease and aging. More than 80 human diseases and syndromes are associated with mitochondrial dysfunction; this book focuses upon diseases linked to mitochondria.

Mitochondrial Mechanisms of Degeneration and Repair in Parkinson`s Disease

Автор: Buhlman Lori M.
Название: Mitochondrial Mechanisms of Degeneration and Repair in Parkinson`s Disease
ISBN: 3319825070 ISBN-13(EAN): 9783319825076
Издательство: Springer
Рейтинг:
Цена: 167700.00 T
Наличие на складе: Есть у поставщика Поставка под заказ.
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.

Mitochondrial Biology and Experimental Therapeutics

Автор: Paulo J. Oliveira
Название: Mitochondrial Biology and Experimental Therapeutics
ISBN: 3030103587 ISBN-13(EAN): 9783030103583
Издательство: Springer
Рейтинг:
Цена: 167700.00 T
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.

Mitochondrial Bioenergetics

Автор: Carlos M. Palmeira; Ant?nio J. Moreno
Название: Mitochondrial Bioenergetics
ISBN: 1493958372 ISBN-13(EAN): 9781493958375
Издательство: Springer
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Цена: 135090.00 T
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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.

Mitochondrial Dynamics and Neurodegeneration

Автор: Bingwei Lu
Название: Mitochondrial Dynamics and Neurodegeneration
ISBN: 9400799470 ISBN-13(EAN): 9789400799479
Издательство: Springer
Рейтинг:
Цена: 174130.00 T
Наличие на складе: Есть у поставщика Поставка под заказ.
Описание: Written by experts, this book offers comprehensive coverage of the mechanisms of mitochondrial dynamics, from basic research on its molecular basis to medical implications, to the direction of future research. Includes numerous color figures and illustrations.


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