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Details recent advances in the pathology of these diseases and implications for treatment
Lysosomal Disorders of the Brain
Recent Advances in Molecular and Cellular Pathogenesis and Treatment
Edited by Frances Platt and Steven Walkley
478 pages
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17 line drawings, 9 halftones and 10 colour figures
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240x168mm
978-0-19-850878-6
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Hardback
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12 February 2004
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This item is printed to order and supplied on a firm sale basis. Items which are printed to order are normally despatched and charged within 5-10 days.
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- A definitive volume on this important group of disorders
- Provides valuable advice on diagnosis and treatment
- Written by international authorities in the field
Lysosomal storage diseases are inherited metabolic disorders characterized by severe pathology, typically involving the brain. Although individually rare, they collectively represent a significant group of diseases that primarily present in early infancy or childhood. In recent years considerable progress has been made in understanding the molecular mechanisms that lead to disordered function of the lysosomal system and to lysosomal storage. Unravelling the basis for these diseases is providing unique insight into the normal biology of cells and pointing the way to the development of therapeutic strategies for their treatment.
Lysosomal
Disorders of Brain details recent advances in the molecular and cellular pathologies of these diseases and in the development of effective therapies. After an overview of the biology of the endosomal-lysosomal system and the types of diseases resulting from defects in this system, the book describes in detail the molecular mechanisms of storage, model systems and pathophysiological mechanisms, and finally, new advances toward treatment. With each chapter written by leading experts in their field, this book will be valuable for scientists and clinicians in helping them understand the role of lysosomes in normal cells and mechanisms underlying these disorders, how they can be diagnosed, and the treatment options that are currently
available.Readership: Research scientists working in the field of genetic brain diseases, neuroscientists interested in the function of the lysosomal/endosomal system of neurons, clinicians specialising in metabolic diseases, neurologists and researchers interested in neurodegenerative disorders.
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Edited by Frances Platt, Glycobiology Institute, Department of Biochemistry, University of Oxford, UK, and Steven Walkley, Department of Neuroscience, Albert Einstein College of Medicine, New York, USA Contributors:
Ljudmila V Borisenko, Dept of Biochemistry, Georg-August-Universitaet, Goettingen, Germany
Dr Terry Butters, Glycobiology Institute, University of Oxford, Oxford UK
Crawley
Dr Alessandra d'Azzo, Dept of Genetics, St Jude Children's Research Hospital, Memphis TN, USA
Thomas Dierks, Dept of Biochemistry, Georg-August-Universitaet, Goettingen, Germany
Dr
Kostantin Dobrenis, Dept of Neuroscience, Albert Einstein College of Medicine, New York, USA
Jens Fey, Dept of Biochemistry, Georg-August-Universitaet, Goettingen, Germany
Dr Andrej Hasilik, Institute of Physiological Chemistry, Philipps-Universitaet, Marburg, Germany
Hers
Prof John Hopwood, Dept of Chemical Pathology, Women's & Children's Hospital, Adelaide, Australia
Dr Yiannis A Ioannou, Dept of Human Genetics, Mt Sinai School of Medicine, New York, USA
Dr Thomas Kolter, Kekule Institute of Organic Chemistry & Biochemistry, Friedrich-Wilhelms-Universititaet, Bonn, Germany
Dr Peter Lemansky, Institute of Physiological Chemistry, Philipps-Universitaet, Marburg, Germany
O Macheleidt, Kekule
Institute of Organic Chemistry & Biochemistry, Friedrich-Wilhelms-Universititaet, Bonn, Germany
Dr Dag Malm, Institute of Clinical Medicine, University of Tromso, Rito, Norway
Dr Fred Maxfield, Dept of Biochemistry, Weill Medical College of Cornell University, New York, USA
Dr Sushmita Mukherjee, Dept of Biochemistry, Weill Medical College of Cornell University, New York, USA
Prof Elizabeth Neufeld, Dept of Biological Chemistry, UCLA, Los Angeles CA, USA
Dr David A Pearce, Center for Aging & Developmental Biology, University of Rochester School of Medicine, Rochester NY, USA
Jianhe Peng, Dept of Biochemistry, Georg-August-Universitaet, Goettingen, Germany
Dr Fran Platt, Glycobiology Institute, University of
Oxford, Oxford UK
Prof Konrad Sandhoff, Kekule Institute of Organic Chemistry & Biochemistry, Friedrich-Wilhelms-Universititaet, Bonn, Germany
Dr Mark S Sands, Washington University School of Medicine, St Louis MO, USA
Bernhard Schmidt, Dept of Biochemistry, Georg-August-Universitaet, Goettingen, Germany
Taylor
Prof Kurt von Figura, Dept of Biochemistry, Georg-August-Universitaet, Goettingen, Germany
Prof Steven Walkley, Dept of Neuroscience, Albert Einstein College of Medicine, New York, USA
Prof Bryan Winchester, Biochemistry, Endocrinology & Metabolism Unit, Institute of Child Health, London, UK
Dr J E Wraith, Willink Biochemical Genetics Unit, Royal Manchester Children's Hospital,
Manchester, UK
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". . . this book is highly recommended . . for those readers wanting to get a clear and straightforward overview of LSDs . . . then this well-written book is a worthwhile investment." - Nature Cell Biology, Vol 6, Number 11
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Hers: Foreword
Platt and Walkley: Preface
Malm: Prologue
Section I: Overview of Lysosomes and Storage Diseases
1: Maxfield and Mukherjee: The endosomal-lysosomal system
2: Platt and Walkley: Lysosomal defects and storage
3: Wraith: Clinical aspects and diagnosis
Section II: Molecular Mechanisms of Storage
4: Winchester: Primary defects in lysosomal enzymes
5: von Figura et al: Defects in lysosomal enzyme modification for catalytic activity
6: Hasilik and Lemansky: Defects in lysosomal enzyme trafficking
7: d'Azzo: Defects in lysosomal enzyme protection: galactosialidosis
8: Sandhoff and Kolter: Defects in activator proteins and other soluble proteins of the lysosome
9: Ioannou: Defects in transmembrane proteins
Section III: Model Systems and Pathophysiological Mechanisms
10: Pearce: Simple non-mammalian systems
11: Hopwood et al: Spontaneous and engineered mammalian storage disease models
12: Walkley: Pathogenic cascades and brain dysfunction
Section IV: Treatment of Storage Diseases
13: Neufeld: Enzyme replacement therapy
14: Dobrenis: Cell-mediated delivery systems
15: Platt and Butters: Inhibition of substrate synthesis: a pharmacological approach for glycosphingolipid storage disease therapy
16: Sands: Gene therapy
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