Lysosomes (Medical Intelligence Unit)

Lysosomes are membrane-surrounded organelles which are present in all animal cells. The importance of this organelle is underlined by an increasing number of human diseases, which are associated with an impaired function of the lysosomal compartment. This book summarizes the current state-of-the art knowledge about this unique organelle. It addresses the biogenesis of this compartment, the transport of lysosomal proteins, the role of the lysosomal membrane in lysosomal stability and transport, the function of lysosomal proteases and hydrolases, lysosomal storage disorders, and new concepts on how to treat these diseases. In addition to these classical topics, new insights into lysosomal functions are covered by chapters dealing with specialized lysosomes involved in bone resorption and plasma membrane repair, the lysosomal transciptome, and proteome and the emerging role of lysosomes in special forms of autophagy. This book will provide readers with a comprehensive overview into how this fascinating organelle works and how research in the field is developing. Cover Page......Page 1 Inside Cover......Page 2 Copyright © 2005......Page 3 Contents......Page 5 Editor, Contributors......Page 9 Preface......Page 11 CHAPTER 1 - History and Morphology of the Lysosome......Page 13 CHAPTER 2 - Transport of Lysosomal Enzymes......Page 29 CHAPTER 3 - Adaptor Proteins in Lysosomal Biogenesis......Page 39 CHAPTER 4 - Lysosomal Membrane Proteins......Page 49 CHAPTER 5 - Lysosomal Proteases: Revival of the Sleeping Beauty......Page 62 CHAPTER 6 - Lysosomal Storage Disorders......Page 72 CHAPTER 7 - Lysosomal Transporters and Associated Diseases......Page 86 CHAPTER 8 - Neuronal Ceroid-Lipofuscinoses......Page 94 CHAPTER 9 - Cholesterol Transport in Lysosomes......Page 112 CHAPTER 10 - Therapy of Lysosomal Storage Diseases......Page 124 CHAPTER 11 - Lysosomal Proteome and Transcriptome......Page 142 CHAPTER 12 - External Lysosomes: The Osteoclast and Its Unique Capacities to Degrade Mineralised Tissues......Page 156 CHAPTER 13 - Membrane Resealing Mediated by Lysosomal Exocytosis......Page 168 CHAPTER 14 - Macroautophagy in Mammalian Cells......Page 178 CHAPTER 15 - Chaperone-Mediated Autophagy......Page 193 Index......Page 206 Back Cover......Page 209 History And Morphology Of The Lysosome / Renate Lüllmann-rauch -- Transport Of Lysosomal Enzymes / Stephan Storch And Thomas Braulke -- Adaptor Proteins In Lysosomal Biogenesis / Peter Schu -- Lysosomal Membrane Proteins / Paul Saftig -- Lysosomal Proteases: Revival Of The Sleeping Beauty / Klaudia Brix -- Lysosomal Storage Disorders / Ole Kristian Greiner-tollersrud And Thomas Berg -- Lysosomal Transporters And Associated Diseases / Frans W. Verheijen And Grazia M.s. Mancini -- Neuronal Ceroid-lipofuscinoses / Jaana Tyynelè -- Cholesterol Transport In Lysosomes / Judith Storch And Sunita R. Cheruku -- Therapy Of Lysosomal Storage Diseases / Ulrich Matzner -- Lysosomal Proteome And Transcriptome / Jobst Landgrebe And Torben Lubke -- External Lysosomes: The Osteoclast And Its Unique Capacities / Vincent Everts And Wouter Beertsen -- Membrane Resealing Mediated By Lysosomal Exocytosis / Norma W. Andrews -- Macroautophagy In Mammalian Cells / Eeva-liisa Eskelinen -- Chaperone-mediated Autophagy / Erwin Knecht And Natalia Salvador. Paul Saftig. Includes Bibliographical References And Index. Lysosomes are membrane-delimited organdíes which occur in all mammalian cells except red blood cells.