Protein Folding Disorders of the Central Nervous System

Jorge Ghiso, Agueda Rostagno;;;

• Publisher: WSPC
• Year: 2017
• ISBN:
  9789813222977
• Topic: Biological Sciences
• Subtopic: Neuroscience
• Index: Biological Sciences

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This exciting new book explores the dark side of the molecular protein assembly bringing an updated view of how failures in the homeostatic mechanisms that efficiently regulate protein folding leads to the accumulation of structurally abnormal pathogenic assemblies, encompassing an emerging group of diseases collectively known as "Protein Folding Disorders." This complex and diverse group of chronic and progressive entities are bridged together by their relationship to structural transitions in the native state of specific proteinaceous components, which for reasons poorly understood, convert into polymeric aggregates that generate poorly soluble tissue deposits and which are considered today the culprit of the disease pathogenesis in their respective diseases. Despite the diversity in the amino acid sequence of the different proteins involved in these heterogeneous disorders, all the pathologic conformers can trigger cascades of events ultimately resulting in cell dysfunction and death with devastating clinical consequences in many of the most precious aspects of human existence including personality, cognition, memory, and skilled movements.

This book, which is composed of a compilation of chapters authored by outstanding and well-published scientists in the respective fields currently performing active investigations at world renowned universities and research centers, focuses on the growing number of diseases associated with protein misfolding in the central nervous system. Individual chapters are dedicated to the most common neurodegenerative diseases associated with protein aggregation/fibrillization focusing on the nature of the pathogenic species and the cellular pathways involved in the molecular pathogenesis of Alzheimer's, Parkinson's, and Huntington's diseases as well as in Amyotrophic Lateral Sclerosis, and Prion disorders. A group of contributions is centered on the current knowledge of the intracellular pathways and subcellular organelles affected by the different disease conditions, while others are focused in the emerging pathogenic role of misfolded subunits assembled into neurotoxic soluble oligomers, and in the novel notion of the transmissibility of the protein misfolded species, an innovative concept until recently only accepted for Prion diseases. Lastly, a different set of chapters is dedicated to the evaluation of novel therapeutic strategies for these devastating diseases.

--> Contents:

• Misfolding, Aggregation, and Amyloid Formation: The Dark Side of Proteins (Agueda Rostagno and Jorge A Ghiso)
• Oligomers at the Synapse: Synaptic Dysfunction and Neurodegeneration (Emily Vogler, Matthew Mahavongtrakul, and Jorge Busciglio)
• Prion-Like Protein Seeding and the Pathobiology of Alzheimer's Disease (Lary C Walker)
• The Tau Misfolding Pathway to Dementia (Alejandra D Alonso, Leah S Cohen, and Viktoriya Morozova)
• The Biology and Pathobiology of α-Synuclein (Joel C Watts, Anurag Tandon, and Paul E Fraser)
• Impact of Loss of Proteostasis on Central Nervous System Disorders (Sentiljana Gumeni, Eleni N Tsakiri, Christina-Maria Cheimonidi, Zoi Evangelakou, Despoina Gianniou, Kostantinos Tallas, Eleni-Dimitra Papanagnou, Aimilia D Sklirou, and Ioannis P Trougakos)
• Protein Misfolding and Mitochondrial Dysfunction in Amyotrophic Lateral Sclerosis (Giovanni Manfredi and Hibiki Kawamata)
• Impact of Mitostasis and the Role of the Anti-Oxidant Responses on Central Nervous System Disorders (Sentiljana Gumeni, Eleni N Tsakiri, Christina-Maria Cheimonidi, Zoi Evangelakou, Despoina Gianniou, Kostantinos Tallas, Eleni-Dimitra Papanagnou, Aimilia D Sklirou, and Ioannis P Trougakos)
• Propagation of Misfolded Proteins in Neurodegeneration: Insights and Cautions from the Study of Prion Disease Prototypes (Robert C C Mercer, Nathalie Daude, and David Westaway)
• Endoplasmic Reticulum Stress Response in Neurodegenerative Diseases (Hyung Don Ryoo)
• Proteomic Analysis of Huntingtin-Associated Proteins Provides Clues to Altered Cell Homeostasis in Huntington's Disease (Naoko Tanese)
• Overcoming the Obstacle of the Blood-Brain Barrier for Delivery of Alzheimer's Disease Therapeutics (Eliezer Masliah and Brian Spencer)
• Immunotherapies for Alzheimer's Disease (Einar M Sigurdsson)
• Role of the Microbiome in Polyphenol Metabolite-Mediated Attenuation of β-Amyloid and Tau Protein Misfolding in Alzheimer's Disease (Jun Wang, Lap Ho, Jeremiah Faith, Kenjiro Ono, Hanna Księżak-Reding, Ali Sharma, Breanna Valcarcel, and Giulio M Pasinetti)

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--> Readership: Graduate students and researchers in neuroscience, neurology, neurobiology and biochemistry. -->
Keywords:Protein Folding;Alzheimer's Disease;Parkinson's Disease;Amyotrophic Lateral Sclerosis;Huntington's Disease;Prion Diseases;Cerebrovascular Amyloidosis;?-Synuclein;Amyloid-?;Tau;Proteostasis;Mitochondria;Endoplasmic Reticulum;Blood-Brain-Barrier;Mitostasis;Polyphenol;Alzheimer ImmunotherapyReview: Key Features:

• The book is a multi-author volume in which each of the contributors is an active investigator and a prominent scientist in the respective fields
• The volume provides a much-needed update of the new findings in the field, compiled together in a single volume
• The book differs from other competing titles by providing in-depth and novel information into different aspects of protein folding disorders. The topics covered range from the structural protein changes associated with conformational disorders, to the involvement of the different subcellular organelles and the novel findings into the respective molecular mechanisms of disease pathogenesis, while providing an insight into some of the most recent therapeutic approaches for these complex disorders

Table of contents

• Cover Page
• Title
• Copyright
• Preface
• List of Contributors
• List of Figures
• List of Tables
• Chapter 1 Misfolding, Aggregation, and Amyloid Formation: The Dark Side of Proteins
• Chapter 2 Oligomers at the Synapse: Synaptic Dysfunction and Neurodegeneration
• Chapter 3 Prion-like Protein Seeding and the Pathobiology of Alzheimer’s Disease
• Chapter 4 The Tau Misfolding Pathway to Dementia
• Chapter 5 The Biology and Pathobiology of α-Synuclein
• Chapter 6 Impact of Loss of Proteostasis on Central Nervous System Disorders
• Chapter 7 Protein Misfolding and Mitochondrial Dysfunction in Amyotrophic Lateral Sclerosis
• Chapter 8 Impact of Mitostasis and the Role of the Anti-oxidant Responses on Central Nervous System Disorders
• Chapter 9 Propagation of Misfolded Proteins in Neurodegeneration: Insights and Cautions from the Study of Prion Disease Prototypes
• Chapter 10 Endoplasmic Reticulum Stress Response in Neurodegenerative Diseases
• Chapter 11 Proteomic Analysis of Huntingtin-Associated Proteins Provides Clues to Altered Cell Homeostasis in Huntington’s Disease
• Chapter 12 Overcoming the Obstacle of the Blood–Brain Barrier for Delivery of Alzheimer’s Disease Therapeutics
• Chapter 13 Immunotherapies for Alzheimer’s Disease
• Chapter 14 Role of the Microbiome in Polyphenol Metabolite-Mediated Attenuation of β-amyloid and tau Protein Misfolding in Alzheimer’s Disease
• Index