Protein Quality Control In Neurodegenerative Diseases

Author : Richard I. Morimoto
Publisher : Springer Science & Business Media
ISBN 13 : 3642279287
Total Pages : 145 pages
Book Rating : 4.87/5 ( download)

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Download or read book Protein Quality Control in Neurodegenerative Diseases written by Richard I. Morimoto and published by Springer Science & Business Media. This book was released on 2012-12-13 with total page 145 pages. Available in PDF, EPUB and Kindle. Book excerpt: The health of the proteome depends upon protein quality control to regulate the proper synthesis, folding, translocation, and clearance of proteins. The cell is challenged constantly by environmental and physiological stress, aging, and the chronic expressions of disease associated misfolded proteins. Substantial evidence supports the hypothesis that the expression of damaged proteins initiates a cascade of molecular events that leads to Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, Huntington's disease, and other diseases of protein conformation.

Author : Richard I. Morimoto
Publisher : Springer
ISBN 13 : 9783642279294
Total Pages : 136 pages
Book Rating : 4.95/5 ( download)

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Download or read book Protein Quality Control in Neurodegenerative Diseases written by Richard I. Morimoto and published by Springer. This book was released on 2012-12-12 with total page 136 pages. Available in PDF, EPUB and Kindle. Book excerpt: The health of the proteome depends upon protein quality control to regulate the proper synthesis, folding, translocation, and clearance of proteins. The cell is challenged constantly by environmental and physiological stress, aging, and the chronic expressions of disease associated misfolded proteins. Substantial evidence supports the hypothesis that the expression of damaged proteins initiates a cascade of molecular events that leads to Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, Huntington's disease, and other diseases of protein conformation.

Author : Uddin, Md. Sahab
Publisher : IGI Global
ISBN 13 : 1799813185
Total Pages : 515 pages
Book Rating : 4.87/5 ( download)

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Download or read book Quality Control of Cellular Protein in Neurodegenerative Disorders written by Uddin, Md. Sahab and published by IGI Global. This book was released on 2020-02-14 with total page 515 pages. Available in PDF, EPUB and Kindle. Book excerpt: Protein misfolding and aggregation are hallmarks of several neurodegenerative proteinopathies. Though multiple factors like aging, oxidative stress, mitochondrial dysfunction, proteotoxic insults, genetic inconsistency, etc. are responsible for the dysfunction of the neuronal protein quality control system, targeting protein quality control has become an auspicious approach to halt the propagation of neurodegeneration. Quality Control of Cellular Protein in Neurodegenerative Disorders provides diverse aspects exploring the role of the protein quality control in neurodegenerative disorders and potential therapeutic strategies to combat the development and propagation of neurodegeneration. Featuring coverage on a broad range of topics such as molecular chaperones, protein misfolding, and stress signaling, this book is ideally designed for neurobiologists, neuropsychologists, neurophysiologists, medical professionals, neuropathologists, researchers, academicians, students, and practitioners engaged in studies of the protein quality control system in neuronal cells.

Author : Cintia Roodveldt
Publisher : Frontiers Media SA
ISBN 13 : 2889453421
Total Pages : 182 pages
Book Rating : 4.29/5 ( download)

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Download or read book Molecular Chaperones and Neurodegeneration written by Cintia Roodveldt and published by Frontiers Media SA. This book was released on 2017-12-06 with total page 182 pages. Available in PDF, EPUB and Kindle. Book excerpt: Molecular chaperones or heat-shock proteins (HSPs) play essential roles in safeguarding structural stability and preventing misfolding and aggregation of proteins, and maintaining the proteome functionality in the cell. For over two decades until the present time, new functions have been discovered and several molecular mechanisms have been elucidated for many chaperones, while the field is being continuously challenged by new open questions. Probably as a consequence of the increasing research on the molecular bases of neurodegenerative diseases, and the realisation that many such disorders are linked to protein misfolding processes, unleashing the roles and mechanisms of chaperones in the context of neurodegeneration has become a prime scientific goal. This e-book contains a diversity of reviews, perspective and original research articles highlighting the importance and potential of this emerging subject.

Author : Martin Beckerman
Publisher :
ISBN 13 : 9783319221182
Total Pages : pages
Book Rating : 4.83/5 ( download)

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Download or read book Fundamentals of Neurodegeneration and Protein Misfolding Disorders written by Martin Beckerman and published by . This book was released on 2015 with total page pages. Available in PDF, EPUB and Kindle. Book excerpt: This unique text introduces students and researchers to the world of misfolded proteins, toxic oligomers, and amyloid assemblages, and the diseases of the brain that result. During the past few years the connections between failures in protein quality control and neurological disorders have been reinforced and strengthened by discoveries on multiple fronts. These findings provide novel insights on how amyloidogenic oligomers and fibrils form, interconvert from one state to another, and propagate from cell to cell and region to region. Starting with protein folding and protein quality control basics, the reader will learn how misfolded proteins can cause diseases ranging from prion diseases to Alzheimer's disease and Parkinson's disease to Huntington's disease, amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Authoritative but written in a clear and engaging style, Fundamentals of Neurodegeneration and Protein Misfolding Disorders addresses one of today's forefront areas of science and medicine. The text emphasizes the new groundbreaking biophysical and biochemical methods that enable molecular-level explorations and the conceptual breakthroughs that result. It contains separate chapters on each of the major disease classes. Special emphasis is placed on those factors and themes that are common to the diseases, especially failures in synaptic transmission, mitochondrial control, and axonal transport; breakdowns in RNA processing; the potential role of environmental factors; and the confounding effects of neuroinflammation. The book is ideal for use in teaching at the advanced undergraduate and graduate levels, and serves as a comprehensive reference for a broad audience of students and researchers in neuroscience, molecular biology, biological physics and biomedical engineering.

Author : Xuekai Zhang
Publisher :
ISBN 13 :
Total Pages : pages
Book Rating : 4.97/5 ( download)

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Download or read book The Role of Chaperone Proteins in Neurodegenerative Diseases written by Xuekai Zhang and published by . This book was released on 2013 with total page pages. Available in PDF, EPUB and Kindle. Book excerpt: Many neurodegenerative diseases are characterized by the accumulation of misfolded proteins that often share common morphological and biochemical features, and can similarly co-localize with several other proteins, including various chaperone proteins. Chaperone proteins, like heat shock protein 27 (HSP27), heme oxygenase 1 (HO-1) and clusterin, have been implicated as potent modulators of misfolded proteins, thus may play important roles in the pathogenesis of neurodegenerative diseases. The present study aims to investigate their roles in the pathogenesis of Frontotemporal lobar degeneration (FTLD), Alzheimer's disease (AD), Parkinson's disease (PD), and Motor neuron disease (MND) by determining their distribution and amount via immunohistochemical staining and western blotting in diseased and control subjects. There were distinct patterns of HSP27 and clusterin immunostaining in different brain regions. For HSP27, patients with AD and FTLD were in general more severely affected than were patients with MND and control subjects. For clusterin, patients with AD and FTLD were more severely affected than control subjects where neurons and glial cells were concerned, while patients with AD and control subjects were more severely affected than those with FTLD where diffuse and cored plaques were concerned. However, there were no obvious differences in the pattern of HO-1 immunostaining in various brain regions in patients with AD or FTLD relative to control subjects. Moreover, there was no association between HSP27, HO-1 and clusterin with disease or histological type, and the 'classic' neuropathological changes in FTLD, AD and MND were not immunoreactive to any of these proteins. There were significant correlations between the degrees of HO-1 and clusterin immunostaining in many brain areas for both AD and FTLD cases, and for all cases overall, but none between HSP27 and clusterin or HSP27 and HO-1. Present results suggest an involvement with ongoing cellular stress, misfolded or unfolded protein accumulation or the deficits/failure of other relevant protein quality control systems, in the pathogenesis of these neurodegenerative diseases. Present work may therefore have implications for the further development of ideas concerning the cause or treatment of neurodegenerative diseases where there is aberrant accumulation of misfolded, aggregated protein, and perhaps for conformational diseases in general. However, there are still many issues remain to be elucidated. Further research aimed at understanding the function and mechanisms of the chaperone system, and other protein quality control mechanisms, in the pathogenesis of neurodegenerative diseases is still needed.

Author : Peter Bross
Publisher : Springer Science & Business Media
ISBN 13 : 1592593941
Total Pages : 317 pages
Book Rating : 4.41/5 ( download)

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Download or read book Protein Misfolding and Disease written by Peter Bross and published by Springer Science & Business Media. This book was released on 2008-02-02 with total page 317 pages. Available in PDF, EPUB and Kindle. Book excerpt: For decades it has been known that structured conformations are important for the proper functioning of most cellular proteins. However, appreciation that protein folding to the functional conformations as well as the structural maintenance of protein molecules are very complex processes has only emerged during the last ten years. The intimate interplay uncovered by this scientific development led us to realize that perturbations of the protein folding process and disturbances of conformational maintenance are major disease mechanisms. This development has given rise to the concept of conformational diseases and the broader signature of protein folding diseases, comprising diseases in which mutations or environmental stresses may result in a partial misfolding that leads then to alternative conformations capable of disturbing cellular processes. This may happen by self-association (aggregation), as in prion and Alzheimer’s diseases, or by incorporation of alternatively folded subunits into structural entities, as in collagen diseases. Another possibility is that folding to the native structure is impaired or abolished, resulting in decreased stea- state levels of the correctly folded protein, as is observed in cystic fibrosis and 1-antitrypsin deficiency, as well as in many enzyme deficiencies. In addition, deficiencies of proteins that are engaged in assisting and supervising protein folding (protein quality control) may impair the folding of many other proteins, resulting in pathological phenotypes. Examples of this are the spastic paraplegia attributable to mutations in mitochondrial protease/chaperone complexes.

Author : Judit Ovádi
Publisher : Springer Science & Business Media
ISBN 13 : 1402094345
Total Pages : 284 pages
Book Rating : 4.47/5 ( download)

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Download or read book Protein folding and misfolding: neurodegenerative diseases written by Judit Ovádi and published by Springer Science & Business Media. This book was released on 2008-12-21 with total page 284 pages. Available in PDF, EPUB and Kindle. Book excerpt: Offering all the latest in the study of neurodegenerative diseases, this book reviews the molecular events initiated by unfolded or misfolded proteins leading to conformational human diseases, especially those found in Parkinson’s and Alzheimer’s diseases.

Author : Michael S. Wolfe
Publisher : Academic Press
ISBN 13 : 0128113057
Total Pages : 560 pages
Book Rating : 4.59/5 ( download)

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Download or read book The Molecular and Cellular Basis of Neurodegenerative Diseases written by Michael S. Wolfe and published by Academic Press. This book was released on 2018-03-29 with total page 560 pages. Available in PDF, EPUB and Kindle. Book excerpt: The Molecular and Cellular Basis of Neurodegenerative Diseases: Underlying Mechanisms presents the pathology, genetics, biochemistry and cell biology of the major human neurodegenerative diseases, including Alzheimer’s, Parkinson’s, frontotemporal dementia, ALS, Huntington’s, and prion diseases. Edited and authored by internationally recognized leaders in the field, the book's chapters explore their pathogenic commonalities and differences, also including discussions of animal models and prospects for therapeutics. Diseases are presented first, with common mechanisms later. Individual chapters discuss each major neurodegenerative disease, integrating this information to offer multiple molecular and cellular mechanisms that diseases may have in common. This book provides readers with a timely update on this rapidly advancing area of investigation, presenting an invaluable resource for researchers in the field. Covers the spectrum of neurodegenerative diseases and their complex genetic, pathological, biochemical and cellular features Focuses on leading hypotheses regarding the biochemical and cellular dysfunctions that cause neurodegeneration Details features, advantages and limitations of animal models, as well as prospects for therapeutic development Authored by internationally recognized leaders in the field Includes illustrations that help clarify and consolidate complex concepts

Author : Dr.Hakim Saboowala
Publisher : Dr.Hakim Saboowala
ISBN 13 :
Total Pages : 70 pages
Book Rating : 4./5 ( download)

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Download or read book Role of Misfolded Proteins in the Pathogenesis of Neurodegenerative Disorders and Challenges impacting the development of Novel Therapies. An Overview. written by Dr.Hakim Saboowala and published by Dr.Hakim Saboowala. This book was released on 2020-11-09 with total page 70 pages. Available in PDF, EPUB and Kindle. Book excerpt: Role of Misfolded Proteins in the Pathogenesis of Neurodegenerative Disorders and Challenges impacting the development of Novel Therapies. An Overview. A hallmark of neurodegenerative proteinopathies is the formation of misfolded protein aggregates that cause cellular toxicity and contribute to cellular proteostatic collapse. Therapeutic targeting of protein misfolding has generated unique challenges for drug discovery and development for several reasons, including: 1)The dynamic nature of the protein species involved, 2)Uncertainty about which forms of a given disease protein such as Monomers, Oligomers, or Insoluble aggregates, are primarily responsible for cellular toxicity, 3)Our still limited understanding about which components of the cellular proteo-static machinery these disease proteins interact with and 4) Lack of well-validated biomarkers for clinical trials. Therapeutic options are currently being explored that target different steps in the production and processing of proteins implicated in neurodegenerative disease, including synthesis, chaperone-assisted folding and trafficking, and degradation via the proteasome and autophagy pathways. Other therapies, like mTOR inhibitors and activators of the heat shock response, can rebalance the entire proteostatic network. Hence an attempt has been made in this E-Booklet to discuss major challenges that impact the development of novel therapies, including incomplete knowledge of druggable disease targets and their mechanism of action as well as a lack of biomarkers to monitor disease progression and therapeutic response. …Dr. H. K. Saboowala. M.B.(Bom) .M.R.S.H.(London)