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. 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. History and Morphology of the Lysosome....Pages 1-16 Transport of Lysosomal Enzymes....Pages 17-26 Adaptor Proteins in Lysosomal Biogenesis....Pages 27-36 Lysosomal Membrane Proteins....Pages 37-49 Lysosomal Proteases....Pages 50-59 Lysosomal Storage Disorders....Pages 60-73 Lysosomal Transporters and Associated Diseases....Pages 74-81 Neuronal Ceroid-Lipofuscinoses....Pages 82-99 Cholesterol Transport in Lysosomes....Pages 100-111 Therapy of Lysosomal Storage Diseases....Pages 112-129 Lysosomal Proteome and Transcriptome....Pages 130-143 External Lysosomes....Pages 144-155 Membrane Resealing Mediated by Lysosomal Exocytosis....Pages 156-165 Macroautophagy in Mammalian Cells....Pages 166-180 Chaperone-Mediated Autophagy....Pages 181-193 Lysosomes are membrane-delimited organdíes which occur in all mammalian cells except red blood cells.
