As 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.

Methods in Toxicology, Volume 2: Mitochondrial Dysfunction provides a source of methods, techniques, and experimental approaches for studying the role of abnormal mitochondrial function in cell injury. The book discusses the methods for the preparation and basic functional assessment of mitochondria from liver, kidney, muscle, and brain; the methods for assessing mitochondrial dysfunction in vivo and in intact organs; and the structural aspects of mitochondrial dysfunction are addressed. The text also describes chemical detoxification and metabolism as well as specific metabolic reactions that are especially important targets or indicators of damage. The methods for measurement of alterations in fatty acid and phospholipid metabolism and for the analysis and manipulation of oxidative injury and antioxidant systems are also considered. The book further tackles additional methods on mitochondrial energetics and transport processes; approaches for assessing impaired function of mitochondria; and genetic and developmental aspects of mitochondrial disease and toxicology. The text also looks into mitochondrial DNA synthesis, covalent binding to mitochondrial DNA, DNA repair, and mitochondrial dysfunction in the context of developing individuals and cellular differentiation. Microbiologists, toxicologists, biochemists, and molecular pharmacologists will find the book invaluable.

Oxidative stress is the result of an imbalance in pro-oxidant/antioxidant homeostasis that leads to the generation of toxic reactive oxygen species. Brain cells are continuously exposed to reactive oxygen species generated by oxidative metabolism, and in certain pathological conditions defense mechanisms against oxygen radicals may be weakened and/or overwhelmed. DNA is a potential target for oxidative damage, and genomic damage can contribute to neuropathogenesis. It is important therefore to identify tools for the quantitative analysis of DNA damage in models on neurological disorders. This book presents detailed information on various neurodegenerative disorders and their connection with oxidative stress. This information will provide clinicians with directions to treat these disorders with appropriate therapy and is also of vital importance for the drug industries for the design of new drugs for treatment of degenerative disorders.* Contains the latest information on the subject of neurodegenerative disorders* Reflects on various factors involved in degeneration and gives suggestions for how to tackle these problems

This book is a printed edition of the Special Issue "Mitochondrial Dysfunction in Ageing and Diseases" that was published in IJMS

Mitochondrial Dysfunction and Nanotherapeutics: Aging, Diseases, and Nanotechnology-Related Strategies in Mitochondrial Medicine provides a comprehensive overview of mitochondrial dysfunction and current strategies for targeting the organelle. Based on the most current research, the editor lined up a team of worldwide experts to cover the most exciting research in the area, considering the impact through the human life span. This book is structured in two parts that provide a good balance of foundational and applied content. Part I deals with an overview of mitochondrial dysfunction and its role in the aging process, including metabolic diseases, neuro-affective and neurodegenerative disorders, sepsis, and toxicological aspects. Part II covers therapeutic substance delivery to mitochondria with a focus on cancer, neurodegenerative diseases, and increasing the bioavailability of natural compounds of interest. Several nanoscale strategies are described. Mitochondrial Dysfunction and Nanotherapeutics: Aging, Diseases, and Nanotechnology-Related Strategies in Mitochondrial Medicine is a complete resource for researchers in this exciting field. Its comprehensive coverage makes this book particularly interesting to bioscience researchers looking to understand the foundations of mitochondrial health throughout the human life span. Additionally, clinician researchers, medical doctors, nutritionists, pharmacologists, and sports scientists may be attracted to the detailed information on the organelle-targeted delivery strategies. - Contains detailed information on mitochondrial dysfunction - Reviews our current understanding of the role of mitochondria in aging - Includes coverage of specific conditions, including sports and affective disorders, among others - Discusses mitochondria targeted delivery of therapeutic compounds

This multidisciplinary book includes current research papers and reviews in the areas of basic neuroscience, neural mechanisms underlying neurodegenerative disorders. It further includes new approaches for neuroprotective treatments, clinical, neurobiological and treatment aspects of psychiatric disorders. The book was conceived as a celebration of the professional life and work of Peter Riederer to mark the occasion of his retirement.

Neurodegenerative diseases, including Alzheimer’s and Parkinson’s disease, are a growing problem across the world’s aging population. Oxidative stress in the brain plays a central role in a common pathophysiology of these diseases. This book presents scientific research on the potential of antioxidant therapy in the prevention and treatment of neurodegenerative disorders. This book outlines the roles of oxidative stress and diabetes mellitus in neurodegeneration, describes the molecular mechanisms of neurodegenerative disorders including the roles of environmental pollutants and inflammatory responses, and explores mitochondrial dysfunction. It then describes the protective abilities of antioxidants – including vitamin D, tocotrienol and coenzyme Q10 – against neurodegeneration. The book demonstrates the therapeutic potential of ketogenic diets, and highlights the roles of medicinal plants, phytopharmaceuticals, traditional medicines and food nutrients in neuroprotection. Key Features: Explains damage caused by numerous neurodegenerative disorders and the possible protection offered by antioxidants and functional foods. Describes molecular mechanisms of neurodegeneration by oxidative stress, advancing age, diabetes and mitochondrial dysfunctions. Demonstrates protection offered by nutraceuticals, antioxidants, botanical extracts and functional foods. The book contains twenty-three chapters divided into six sections written by leading researchers. This book is essential reading for health professionals, dietitians, food and nutrition scientists and anyone wanting to improve their knowledge of etiology of neurodegenerative diseases.

This monograph describes the progress in neuropathological HD research made during the last century, the neuropathological hallmarks of HD and their pathogenic relevance. Starting with the initial descriptions of the progressive degeneration of the striatum as one of the key events in HD, the worldwide practiced Vonsattel HD grading system of striatal neurodegeneration will be outlined. Correlating neuropathological data with results on the functional neuroanatomy of the human brain, subsequent chapters will highlight recent HD findings: the neuronal loss in the cerebral neo-and allocortex, the neurodegeneration of select thalamic nuclei, the affection of the cerebellar cortex and nuclei, the involvement of select brainstem nuclei, as well as the pathophysiological relevance of these pathologies for the clinical picture of HD. Finally, the potential pathophysiological role of neuronal huntingtin aggregations and the most important and enduring challenges of neuropathological HD research are discussed.

The editor of this volume, having research interests in the field of ROS production and the damage to cellular systems, has identified a number of enzymes showing ·OH scavenging activities details of which are anticipated to be published in the near future as confirmatory experiments are awaited. It is hoped that the information presented in this book on NDs will stimulate both expert and novice researchers in the field with excellent overviews of the current status of research and pointers to future research goals. Clinicians, nurses as well as families and caregivers should also benefit from the material presented in handling and treating their specialised cases. Also the insights gained should be valuable for further understanding of the diseases at molecular levels and should lead to development of new biomarkers, novel diagnostic tools and more effective therapeutic drugs to treat the clinical problems raised by these devastating diseases.

In October 1995, the 1st Colloquium on Mitochondria and Myopathies in Halle/Saale was organized in Halle/Saale by the editors of this focused issue. The meeting took up what might be called an East German tradition: from 1976 to 1990 Andreas Schmidt organized seven clinically orientated Colloquia on Myology in Jena, and from 1974 to 1990 a series of twelve Colloquia on Mitochondria focused on basic research aspects was arranged by Wolfgang Kunz in Magdeburg. At those meetings, East Germany was a mediator between East European, West European and American scientists. In continuation of this tradition, scientists from more than 17 countries working on mitochondria as neurologists, biochemists, geneticists, or as physiologists came to Halle. The title of the colloquium indicated the combination of both basic and clinical mitochondrial research. The most important contributions of this meeting are now published in this focused issue. We thank all authors for their patience and cooperation that have made it possible to produce this unique collection of papers representing current knowledge on detection of mitochondrial causes of diseases. We especially thank Prof. N.S. Dhalla for making it possible to publish these contributions together in this focused issue and also as a hard-cover book.