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Glutamine Repeats and Neurodegenerative Diseases

Glutamine Repeats and Neurodegenerative Diseases
Author: Peter S. Harper
Publisher: Oxford University Press, USA
Total Pages: 352
Release: 2001
Genre: Medical
ISBN:

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This book focuses on the discovery of a common genetic basis for a group of inherited neurological disorders, including Huntington's Disease, spino-bulbar atrophy and a series of hereditary ataxias. This shared molecular background and other similarities have led to the development of theoretical models for the pathogenesis of these diseases. It is now also clear that the mechanisms involved are likely to be of more general relevance, outside of this particular group of disorders, with implications for other neurodegenerative processes such as those involved in Alzheimer's, Parkinson's and Prion diseases. The book is an edited and updated compilation evolving from a Royal Society discussion meeting.


Polyglutamine Disorders

Polyglutamine Disorders
Author: Clévio Nóbrega
Publisher: Springer
Total Pages: 467
Release: 2018-02-09
Genre: Medical
ISBN: 3319717790

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This book provides a cutting-edge review of polyglutamine disorders. It primarily focuses on two main aspects: (1) the mechanisms underlying the pathologies’ development and progression, and (2) the therapeutic strategies that are currently being explored to stop or delay disease progression. Polyglutamine (polyQ) disorders are a group of inherited neurodegenerative diseases with a fatal outcome that are caused by an abnormal expansion of a coding trinucleotide repeat (CAG), which is then translated in an abnormal protein with an elongated glutamine tract (Q). To date, nine polyQ disorders have been identified and described: dentatorubral-pallidoluysian atrophy (DRPLA); Huntington’s disease (HD); spinal–bulbar muscular atrophy (SBMA); and six spinocerebellar ataxias (SCA 1, 2, 3, 6, 7, and 17). The genetic basis of polyQ disorders is well established and described, and despite important advances that have opened up the possibility of generating genetic models of the disease, the mechanisms that cause neuronal degeneration are still largely unknown and there is currently no treatment available for these disorders. Further, it is believed that the different polyQ may share some mechanisms and pathways contributing to neurodegeneration and disease progression.


Neuropathology of Neurodegenerative Diseases

Neuropathology of Neurodegenerative Diseases
Author: Gabor G. Kovacs
Publisher: Cambridge University Press
Total Pages: 320
Release: 2017-12-13
Genre: Medical
ISBN: 1316337650

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This practical guide to the diagnosis of neurodegenerative diseases discusses modern molecular techniques, morphological classification, fundamentals of clinical symptomology, diagnostic pitfalls and immunostaining protocols. It is based on the proteinopathy concept of neurodegenerative disease, which has influenced classification and provides new strategies for therapy. Numerous high-quality images, including histopathology photomicrographs and neuroradiology scans, accompany the description of morphologic alterations and interpretation of immunoreactivities. Diagnostic methods and criteria are placed within recent developments in neuropathology, including the now widespread application of immunohistochemistry. To aid daily practice, the guide includes diagnostic algorithms and offers personal insights from experienced experts in the field. Special focus is given to the way brain tissue should be handled during diagnosis. This is a must-have reference for medical specialists and specialist medical trainees in the fields of pathology, neuropathology and neurology working with neuropathologic features of neurodegenerative diseases.


Molecular Mechanisms of Neurodegenerative Diseases

Molecular Mechanisms of Neurodegenerative Diseases
Author: Marie-Francoise Chesselet
Publisher: Springer Science & Business Media
Total Pages: 416
Release: 2000-10-19
Genre: Medical
ISBN: 1592590063

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With the unprecedented identification of new mutation mechanisms in neurodegenerative diseases and the emergence of common mechanisms among diseases that were once considered unrelated, neurobiologists are poised for the development of new therapies based on high throughput screenings and a better understanding of the molecular and cellular mechanisms leading to neurodegeneration. In Molecular Mechanisms of Neurodegenerative Diseases, Marie-Francoise Chesselet, MD, PhD, and a panel of leading researchers and neurologists from industry and academia critically review the most recent advances from different yet complementary points of view. Focusing on Alzheimer's, Parkinson's, and CAG triplet repeat diseases, the authors show how studies of cellular and genetically engineered animal models have enhanced our understanding of the molecular mechanisms of neurodegenerative diseases and may lead to the development of new therapeutics. Topics include the role of Ab toxicity, glial cells, and inflammation in Alzheimer's disease; the formation of abnormal protein fragments across several diseases, the impact of dopamine and mitochondrial dysfunction on neurodegeneration; and the potential of genetics to identify the molecular mechanisms of neurodegenerative diseases. Authoritative and insightful, Molecular Mechanisms of Neurodegenerative Diseases synthesizes the novel ideas and concepts now emerging to create a fresh understanding of neurodegenerative disorders, one that promises to lead to powerful new therapies that prevent, delay the onset, slow the progression, or even cure these cruel diseases.


The Neuropathology of Huntington’s Disease: Classical Findings, Recent Developments and Correlation to Functional Neuroanatomy

The Neuropathology of Huntington’s Disease: Classical Findings, Recent Developments and Correlation to Functional Neuroanatomy
Author: Udo Rüb
Publisher: Springer
Total Pages: 154
Release: 2015-09-29
Genre: Medical
ISBN: 331919285X

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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.


Neuronal Degeneration and Regeneration: From Basic Mechanisms to Prospects for Therapy

Neuronal Degeneration and Regeneration: From Basic Mechanisms to Prospects for Therapy
Author: F.W. Van Leeuwen
Publisher: Elsevier
Total Pages: 551
Release: 1998-11-09
Genre: Psychology
ISBN: 008086242X

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This book is the result of the 20th International Summer School in Brain Research, organized in August 1997 in Amsterdam, by the Netherlands Institute for Brain Research at the Royal Netherlands Academy of Sciences. It is the first book that provides a complete overview of the field of neurodegeneration and regeneration including spinal cord injury, neurodegenerative diseases and therapy. Divided into five sections, the first two sections give an overview of fundamental research on nerve cell death, neuronal survival, neurite outgrowth and guidance. Extensive attention is given to the role of neurotrophins, their receptor tyrosine kinases and cell-adhesion molecules in development and regeneration of the nervous system. The third section of the book is devoted to research involving human neurodegenerative diseases and emerging treatment strategies. Section four focusses on recent advances in the understanding of pathophysiological mechanisms underlying neurodegenerative diseases, including Alzheimer's, Parkinson's and Huntington's diseases along with prion diseases. Novel insights into the neuropathological hallmarks of these diseases, as well as into transgenic animal models, the involvement of environmental factors, and genomic and mRNA changes that can cause neurodegeneration. The final section of this volume reveals recent developments in the use of cell and gene therapy to treat neurodegenerative disease and lesion-related deficits. Implantation of genetically modified cells, direct gene transfer with viral vectors and the first clinical trials with encapsulated genetically modified cells in patients suffering from amyotrophic lateral scelerosis are examples of new therapeutic strategies treating neurodegenerative diseases. The book is of particular interest to departments of neuroscience, neurological clinics and departments, the pharmalogical industry and medical libraries.


Neurodegenerative Diseases

Neurodegenerative Diseases
Author: Nagehan Ersoy Tunalı
Publisher: BoD – Books on Demand
Total Pages: 180
Release: 2021-01-20
Genre: Science
ISBN: 1838801499

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Neurodegenerative diseases represent a very large group of heterogeneous disorders affecting specific subtypes of neurons in the brain. This book contributes insight both to the awareness of the brain and its neurodegenerative states. The chapters present current knowledge regarding genetics, molecular mechanisms, and new therapeutic strategies against neurodegenerative disorders. The book is intended to serve as a source to aid clinicians and researchers in the field, and also life science readers to increase their understanding and awareness of the clinical correlations, genetic aspects, neuropathological findings, and current therapeutic interventions in neurodegenerative diseases. I believe that this book will enlighten the curiosity for neurodegeneration and also encourage researchers to work on potentially effective molecular therapies for still mysterious neurodegenerative disorders.