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Author: Mathias Jucker
Publisher: Springer Science & Business Media
ISBN: 3642354912
Category : Medical
Languages : en
Pages : 163
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Book Description
The misfolding and aggregation of specific proteins is an early and obligatory event in many of the age-related neurodegenerative diseases of humans. The initial cause of this pathogenic cascade and the means whereby disease spreads through the nervous system, remain uncertain. A recent surge of research, first instigated by pathologic similarities between prion disease and Alzheimer’s disease, increasingly implicates the conversion of disease-specific proteins into an aggregate-prone b-sheet-rich state as the prime mover of the neurodegenerative process. This prion-like corruptive protein templating or seeding now characterizes such clinically and etiologically diverse neurological disorders as Alzheimer ́s disease, Parkinson’s disease, Huntington’s disease, amyotrophic lateral sclerosis, and frontotemporal lobar degeneration. Understanding the misfolding, aggregation, trafficking and pathogenicity of the affected proteins could therefore reveal universal pathomechanistic principles for some of the most devastating and intractable human brain disorders. It is time to accept that the prion concept is no longer confined to prionoses but is a promising concept for the understanding and treatment of a remarkable variety of diseases that afflict primarily our aging society.
Author: Mathias Jucker
Publisher: Springer Science & Business Media
ISBN: 3642354912
Category : Medical
Languages : en
Pages : 163
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Book Description
The misfolding and aggregation of specific proteins is an early and obligatory event in many of the age-related neurodegenerative diseases of humans. The initial cause of this pathogenic cascade and the means whereby disease spreads through the nervous system, remain uncertain. A recent surge of research, first instigated by pathologic similarities between prion disease and Alzheimer’s disease, increasingly implicates the conversion of disease-specific proteins into an aggregate-prone b-sheet-rich state as the prime mover of the neurodegenerative process. This prion-like corruptive protein templating or seeding now characterizes such clinically and etiologically diverse neurological disorders as Alzheimer ́s disease, Parkinson’s disease, Huntington’s disease, amyotrophic lateral sclerosis, and frontotemporal lobar degeneration. Understanding the misfolding, aggregation, trafficking and pathogenicity of the affected proteins could therefore reveal universal pathomechanistic principles for some of the most devastating and intractable human brain disorders. It is time to accept that the prion concept is no longer confined to prionoses but is a promising concept for the understanding and treatment of a remarkable variety of diseases that afflict primarily our aging society.
Author: Jesus Avila
Publisher: Frontiers E-books
ISBN: 288919261X
Category : Medicine (General)
Languages : en
Pages : 114
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Book Description
Neurofibrillary tangles (NFTs) composed of intracellular aggregates of tau protein are a key neuropathological feature of Alzheimer’s Disease (AD) and other neurodegenerative diseases, collectively termed tauopathies. The abundance of NFTs has been reported to correlate positively with the severity of cognitive impairment in AD. However, accumulating evidences derived from studies of experimental models have identified that NFTs themselves may not be neurotoxic. Now, many of tau researchers are seeking a “toxic” form of tau protein. Moreover, it was suggested that a “toxic” tau was capable to seed aggregation of native tau protein and to propagate in a prion-like manner. However, the exact neurotoxic tau species remain unclear. Because mature tangles seem to be non-toxic component, “tau oligomers” as the candidate of “toxic” tau have been investigated for more than one decade. In this topic, we will discuss our consensus of “tau oligomers” because the term of “tau oligomers” [e.g. dimer (disulfide bond-dependent or independent), multimer (more than dimer), granular (definition by EM or AFM) and maybe small filamentous aggregates] has been used by each researchers definition. From a biochemical point of view, tau protein has several unique characteristics such as natively unfolded conformation, thermo-stability, acid-stability, and capability of post-translational modifications. Although tau protein research has been continued for a long time, we are still missing the mechanisms of NFT formation. It is unclear how the conversion is occurred from natively unfolded protein to abnormally mis-folded protein. It remains unknown how tau protein can be formed filaments [e.g. paired helical filament (PHF), straight filament and twisted filament] in cells albeit in vitro studies confirmed tau self-assembly by several inducing factors. Researchers are still debating whether tau oligomerization is primary event rather than tau phosphorylation in the tau pathogenesis. Inhibition of either tau phosphorylation or aggregation has been investigated for the prevention of tauopathies, however, it will make an irrelevant result if we don’t know an exact target of neurotoxicity. It is a time to have a consensus of definition, terminology and methodology for the identification of “tau oligomers”.
Author:
Publisher: Academic Press
ISBN: 0128200030
Category : Science
Languages : en
Pages : 400
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Book Description
Prions and Neurodegenerative Diseases, Volume 172, in the Progress in Molecular Biology and Translational Science series, provides the most topical, informative and exciting monographs available on a wide variety of research topics. The series includes in-depth knowledge on the molecular biological aspects of organismal physiology, with this release including chapters on Cell-free amplification of prions: where do we stand? Transgenic mouse models for the study of prion diseases and much more. - Includes comprehensive coverage of molecular biology - Presents ample use of tables, diagrams, schemata and color figures to enhance the reader's ability to rapidly grasp the information provided - Contains contributions from renowned experts in the field - Includes comprehensive coverage of molecular biology - Presents ample use of tables, diagrams, schemata and color figures to enhance the reader's ability to rapidly grasp the information provided - Contains contributions from renowned experts in the field
Author: A. Zara Herskovits
Publisher: Elsevier Health Sciences
ISBN: 0323762697
Category : Medical
Languages : en
Pages : 161
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Book Description
This issue of Clinics in Laboratory Medicine, guest edited by Dr. A. Zara Herskovits, will cover Laboratory Testing for Neurologic Disorders. This issue is one of four selected each year by our Editor-in-Chief, Dr. Milenko Jovan Tanasijevic. Topics discussed in this issue will include: molecular approach to diagnostic testing for children with developmental delay and congenital anomalies, proteopathic and seeding assays (such as RT-QUIC), genetic testing for ALS and FTD, Diagnostic and prognostic testing for Alzheimer's disease, confounds in the interpretation of paraneoplastic antibody panels, Review of neurologic disease sendout testing at an academic medical center, development of new diagnostic tests for neurologic disorders, assuring quality in laboratory testing for sendout reference tests, diagnostic testing for patients with spinal muscular atrophy, among others.
Author: Diana Fernandes Lázaro
Publisher: Frontiers Media SA
ISBN: 2889635074
Category :
Languages : en
Pages : 158
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Book Description
This eBook is a collection of articles from a Frontiers Research Topic. Frontiers Research Topics are very popular trademarks of the Frontiers Journals Series: they are collections of at least ten articles, all centered on a particular subject. With their unique mix of varied contributions from Original Research to Review Articles, Frontiers Research Topics unify the most influential researchers, the latest key findings and historical advances in a hot research area! Find out more on how to host your own Frontiers Research Topic or contribute to one as an author by contacting the Frontiers Editorial Office: frontiersin.org/about/contact.
Author: Victor Chechik
Publisher: Royal Society of Chemistry
ISBN: 1788013883
Category : Science
Languages : en
Pages : 187
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Book Description
Electron Paramagnetic Resonance (EPR) applications remain very significant in modern analytical science and this volume compiles critical coverage of developments in the recent literature by a handpicked group of researchers at the cutting-edge of the field. The topics covered in this volume describe contrasting types of EPR application, including light induced hyperpolarization and disordered proteins to spin labels and nanomaterials. Providing a snap shot of the area, this book is a useful addition to any library supporting this research.
Author: Vijay Kumar
Publisher: Academic Press
ISBN: 0128204400
Category : Medical
Languages : en
Pages : 272
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Book Description
Aggregates of the TAR DNA binding protein 43 (TDP-43), are hallmark features of the neurodegenerative diseases Amyotrophic Lateral Sclerosis (ALS) and frontotemporal dementia (FTD), with overlapping clinical, genetic and pathological features. TDP-43 and Neurodegeneration: From Bench to Bedside summarizes new findings in TDP-43 pathobiology and proteinopathies. The book summarizes TDP-43's structure, function, biology, misfolding, aggregation, pathogenesis and therapeutics. It includes autophagy-mediated therapy, role of stress granule, novel genetic, cell culture-based models, systems biology for precision medicine, development of stem cells and mechanism-based therapies that can target ALS and other related neurodegenerative diseases. This book is written for neuroscientists, neurologists, clinicians, advanced graduate students, drug discovery researchers, as well as cellular and molecular biologists involved in ALS, motor neuron disease (MND) and other neurodegenerative disorders. - Reviews TDP-43 structure, folding, function, and pathology - Identifies TDP-43 role in ALS, FTP, and other neurodegenerative diseases - Presents a systems and precision biology perspective of TDP-43 - Discusses therapeutics of TDP-43 proteinopathies - Translates bench research to application bedside
Author: Stanley B. Prusiner
Publisher: Yale University Press
ISBN: 0300199260
Category : Science
Languages : en
Pages : 445
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Book Description
In this “utterly fascinating” science memoir, the Nobel Prize–winning author chronicles his revolutionary discovery of a major cause of brain diseases (The New York Times). In 1997, Stanley B. Prusiner received the Nobel Prize in Physiology or Medicine for his research on devastating brain diseases. That he was the award’s sole recipient was entirely appropriate. His struggle to identify the agent responsible for scrapie and mad cow disease in animals, and Creutzfeldt-Jakob disease in humans, had been waged largely alone, and in some cases, in the face of strenuous opposition. In Madness and Memory, Prusiner recounts the journey to his remarkable discovery of prions—infectious proteins that replicate and cause disease, but surprisingly contain no genetic material. Along the way, he sheds light on the world of contemporary science and the meticulousness and perseverance it requires. Through his telling, the agony and triumph of years of research comes to life, along with fascinating portraits of fellow scientists racing to make breakthrough discoveries. Prusiner’s hypothesis, once considered heresy, now stands as accepted science and provides a path toward conquering such pervasive scourges as Alzheimer’s, Parkinson’s, Lou Gehrig’s, and others diseases. “Madness and Memory is the story of one of the most important discoveries in recent medical history, and it is also a vivid and compelling portrait of a life in science.” —Oliver Sacks, MD, neurologist and author of Hallucinations
Author:
Publisher: Elsevier
ISBN: 0123864577
Category : Science
Languages : en
Pages : 4376
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Book Description
Pathobiology of Human Disease bridges traditional morphologic and clinical pathology, molecular pathology, and the underlying basic science fields of cell biology, genetics, and molecular biology, which have opened up a new era of research in pathology and underlie the molecular basis of human disease. The work spans more than 48 different biological and medical fields, in five basic sections: Human - Organ Systems - Molecular Pathology/Basic Mechanisms of Diseases - Animal Models/Other Model Systems - Experimental Pathology - Clinical Pathology Each article provides a comprehensive overview of the selected topic to inform a broad spectrum of readers from research professionals to advanced undergraduate students. - Reviews quantitative advances in the imaging and molecular analysis of human tissue, new microarray technologies for analysis of genetic and chromosomal alterations in normal and diseased cells and tissues, and new transgenic models of human disease using conditional, tissue-specific gene targeting - Articles link through to relevant virtual microscopy slides, illustrating side-by-side presentation of "Normal" and "Disease" anatomy and histology images - Fully-annotated with many supplementary full color images, graphs, tables, and video files linked to data sets and to live references, enabling researchers to delve deeper and visualize solutions
Author: Leonard F. M. Scinto
Publisher: Springer Science & Business Media
ISBN:
Category : Medical
Languages : en
Pages : 384
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Book Description
The three major approaches to diagnosis of AD -- radiological, biological, and neurophysiological -- are discussed in detail with chapters highlighting the most promising technologies within these approaches. The leading authors, all of whom are intimately involved with these emerging technologies, have developed this as an essential reference for neuropathologists, clinicians and researchers of Alzheimer's disease.
