Lysosomal Disorders of the Brain: Recent Advances in Molecular and Cellular Pathogenesis and…

Hardcover | February 2, 2005

EditorFrances Platt, Steven Walkley

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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 yearsconsiderable 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 thedevelopment 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, thebook 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 understandthe role of lysosomes in normal cells and mechanisms underlying these disorders, how they can be diagnosed, and the treatment options that are currently available.

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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 yearsconsiderable progress has been made in understandin...

Frances Platt is at Glycobiology Institute, Department of Biochemistry, University of Oxford, UK. Steven Walkley is in the Department of Neurosciences, Pathology and Neurology, Albert Einstein College of Medicine, New York, USA.

other books by Frances Platt

Lysosomes and lysosomal Diseases
Lysosomes and lysosomal Diseases

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Format:HardcoverDimensions:476 pages, 9.45 × 6.61 × 1.2 inPublished:February 2, 2005Publisher:Oxford University PressLanguage:English

The following ISBNs are associated with this title:

ISBN - 10:0198508786

ISBN - 13:9780198508786

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Table of Contents

Hers: ForewordPlatt and Walkley: PrefaceMalm: PrologueSection I: Overview of Lysosomes and Storage Diseases1. Maxfield and Mukherjee: The endosomal-lysosomal system2. Platt and Walkley: Lysosomal defects and storage3. Wraith: Clinical aspects and diagnosisSection II: Molecular Mechanisms of Storage4. Winchester: Primary defects in lysosomal enzymes5. von Figura et al: Defects in lysosomal enzyme modification for catalytic activity6. Hasilik and Lemansky: Defects in lysosomal enzyme trafficking7. d'Azzo: Defects in lysosomal enzyme protection: galactosialidosis8. Sandhoff and Kolter: Defects in activator proteins and other soluble proteins of the lysosome9. Ioannou: Defects in transmembrane proteinsSection III: Model Systems and Pathophysiological Mechanisms10. Pearce: Simple non-mammalian systems11. Hopwood et al: Spontaneous and engineered mammalian storage disease models12. Walkley: Pathogenic cascades and brain dysfunctionSection IV: Treatment of Storage Diseases13. Neufeld: Enzyme replacement therapy14. Dobrenis: Cell-mediated delivery systems15. Platt and Butters: Inhibition of substrate synthesis: a pharmacological approach for glycosphingolipid storage disease therapy16. Sands: Gene therapy