Protein folding and misfolding : neurodegenerative diseases / edited by Judit Ovádi, Ferenc Orosz.

Format:

Book

Contributor:

Ovádi, Judit, 1941-

Orosz, Ferenc.

Series:

Focus on structural biology ; v. 7.

Focus on structural biology ; v. 7

Language:

English

Subjects (All):

Protein folding.

Proteins--Denaturation.

Proteins.

Nervous system--Degeneration--Pathophysiology.

Nervous system.

Protein Folding.

Protein Conformation.

Protein Denaturation.

Neurodegenerative Diseases--physiopathology.

Nervous system--Degeneration.

Medical Subjects:

Protein Folding.

Protein Conformation.

Protein Denaturation.

Neurodegenerative Diseases--physiopathology.

Physical Description:

xiii, 277 pages : illustrations (some color) ; 24 cm.

Place of Publication:

[Dordrecht ;] [London] : Springer, [2009]

Summary:

The worldwide increasing age of populations brought the neurodegenerative diseases into the focus of interest. A number of the diverse human neurodegenerative diseases are now recognized as conformational diseases frequently caused by aggregations of unfolded or misfolded proteins. Knowledge on the intrinsically unstructured proteins, a new family of gene products as well as on the misfolded proteins produced by genetic mutation or environmental effects has been extensively accumulated in the past years. These proteins frequently cause proteolytic stress and/ or enter into aberrant, non-physiological protein-protein interactions leading to sequestration of protein aggregates which are assemblies of many not-yet-identified components in addition to the deposition of well-characterized misfolded peptides and proteins such as β-amyloid, tau, α-synuclein and polyglutamine containing proteins. These protein assemblies display diverse ultrastructures such aggresomes, fibers, oligomers or amorphous structures, however, the nature of these species concerning their cytoprotective or cytotoxic effects has not been clarified yet.

The main focus of this volume is to review the molecular events initiated by unfolded or misfolded proteins leading to conformational human diseases, with special emphasis on the macromolecular homo- and heteroassociations of the malfolded proteins into characteristic ultrastructures found primarily in Parkinson's and Alzheimer's diseases. This book reviews the structural knowledge accumulated for well-studied and for newly discovered proteins involved in paradigmatic conformational disorders with the aim to broaden our understanding of the pathomechanisms of neurodegeneration, which is crucial for finding effective therapeutic interventions that could prevent or circumvent the development of neurodegenerative disorders in humans.

Contents:

1 Structural Disorder and Its Connection with Misfolding Diseases / Veronika Csizmok, Peter Tompa 1

2 Intrinsic Disorder in Proteins Associated with Neurodegenerative Diseases / Vladimir N. Uversky 21

3 Dynamic Role of Ubiquitination in the Management of Misfolded Proteins Associated with Neurodegenerative Diseases / Esther S.P. Wong, Jeanne M.M. Tan, Kah-Leong Lim 77

4 Protein Misfolding and Axonal Protection in Neurodegenerative Diseases / Haruhisa Inoue, Takayuki Kondo, Ling Lin, Sha Mi, Ole Isacson, Ryosuke Takahashi 97

5 Endoplasmic Reticulum Stress in Neurodegeneration / Jeroen J.M. Hoozemans, Wiep Scheper 111

6 Involvement of Alpha-2 Domain in Prion Protein Conformationally-Induced Diseases / Luisa Ronga, Pasquale Palladino, Ettore Benedetti, Raffaele Ragone, Filomena Rossi 133

7 Synuclein Structure and Function in Parkinson's Disease / David Eliezer 159

8 Inhibition of α-Synuclein Aggregation by Antioxidants and Chaperones in Parkinson's Disease / Jean-Christophe Rochet, Fang Liu 175

9 Novel Proteins in α-Synucleinopathies / Christine Lund Kragh, Poul Henning Jensen 207

10 TPPP/p25: A New Unstructured Protein Hallmarking Synucleinopathies / Ferenc Orosz, Attila Lehotzky, Judit Olah, Judit Ovadi 225

11 Protein-Based Neuropathology and Molecular Classification of Human Neurodegenerative Diseases / Gabor G. Kovacs, Herbert Budka 251.

Notes:

Includes bibliographical references and index.

ISBN:

9781402094330

1402094337

OCLC:

276224601