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Preface

History and Morphology of the Lysosome

(16)

Renate Lullmann-Rauch

Morphology of Lysosomes

(9)

History of the Lysosome Concept

(6)

Transport of Lysosomal Enzymes

(10)

Stephan Storch

Thomas Braulke

Synthesis and Modifications of Soluble Lysosomal Proteins

(1)

Formation of Mannose 6-Phosphate Recognition Marker

(1)

Mannose 6-Phosphate Receptors

(1)

Signal Structure-Dependent Trafficking of MPRs

(1)

Sorting Functions of Mannose 6-Phosphate Receptors

(1)

Delivery to the Endosomal/Lysosomal Compartment

(1)

Mannose 6-Phosphate Receptor-Independent Pathways

(1)

Perspectives

(5)

Adaptor Proteins in Lysosomal Biogenesis

(10)

Peter Schu

Heterotetrameric Adaptor-Protein Complexes

(2)

Membrane Binding of Adaptor-Proteins

(1)

Adaptor-Protein Sorting Pathways

(2)

Coadaptor Proteins

(5)

Lysosomal Membrane Proteins

(13)

Paul Saftig

LAMPs and LIMPs as Major Components of the Lysosomal Membrane

(1)

LAMP-Deficient Mice

(2)

Intracellular Trafficking of the Major Lysosomal Membrane Glycoproteins

(1)

Less Abundant Integral Components of the Lysosomal Membrane

(3)

Perspectives

(4)

Lysosomal Proteases: Revival of the Sleeping Beauty

(10)

Klaudia Brix

Expression and Distribution of Lysosomal Proteases

(1)

Importance of the in Situ Determination of Proteolytic Activities

(1)

Functions of Lysosomal Proteases

(7)

Lysosomal Storage Disorders

(14)

Ole Kristian Greiner-Tollersrud

Thomas Berg

The Lysosomal Storage Disorders

(1)

Defects in Glycan Degradation

(1)

Defects in Glycoprotein Degradation (Glycoproteinoses)

(2)

Defects in Glycolipid Degradation

(2)

Degradation of GM1 Ganglioside

(1)

Degradation of Sulfatide

(1)

Degradation of Globotriaosylceramide

(1)

Defects in Glycosaminoglycan Degradation (Mucopolysaccharidoses)

(2)

Defects in Glycogen Degradation

(1)

Defects in Lipid Degradation

(1)

Defects in Protein Degradation

(1)

Defects in Lysosomal Transporters

(1)

Defects in Trafficking

(5)

Lysosomal Transporters and Associated Diseases

(8)

Frans W. Verheijen

Grazia M.S. Mancini

The Lysosomal Membrane and Storage Diseases

(2)

Cystinosin, CTNS and Cystinosis

(1)

Sialin, SLC17A5 and Sialic Acid Storage Disease

(1)

CLN3 and Batten Disease (Juvenile Ceroid Lipofuscinosis)

(1)

NPC1 and Niemann-Pick Disease

(1)

SLC36A1 Encoding LYAAT-1, a Transporter for Small Neutral Amino Acids

(1)

MCOLN1, Mucolipin and Mucolipidosis Type IV

(1)

TCIRG-1 and CICN7, A3 Subunit ATPase and Chloride Channel 7, Lysosomal Acidification Disorder, Infantile Malignant Osteopetrosis (Albers-Schonberg Disease)

(3)

Neuronal Ceroid-Lipofuscinoses

(18)

Jaana Tyynela

Classification and Genetics

(2)

Clinical Characteristics

(1)

Neuronal Degeneration

(2)

Neuronal Storage Material

(1)

Palmitoyl-Protein Thioesterase 1 (PPT1)

(2)

Tripeptidyl Peptidase I (TPPI)

(1)

Cathepsin D

(1)

CLN3 Protein

(1)

CLN5, CLN6 and CLN8 Proteins

(1)

Animal Models

(2)

Pathogenetic Considerations

(7)

Cholesterol Transport in Lysosomes

100

(12)

Judith Storch

Sunita R. Cheruku

Intracellular Cholesterol Transport

100

(1)

Endogenously Synthesized Cholesterol Transport

101

(1)

Exogenously Derived Cholesterol Transport

102

(2)

Mechanism of Cholesterol Transport from Lysosomes

104

(1)

Lysosomal Cholesterol Transport Candidates: Niemann-Pick C Disease

104

(1)

NPC1

105

(1)

NPC2

106

(6)

Therapy of Lysosomal Storage Diseases

112

(18)

Ulrich Matzner

Lysosomal Storage Diseases

112

(4)

Enzyme Replacement Therapy

116

(1)

Transplantation Therapy

117

(3)

In Vivo Gene Therapy

120

(2)

Enzyme Enhancement Therapy

122

(1)

Substrate Reduction Therapy

123

(7)

Lysosomal Proteome and Transcriptome

130

(14)

Jobst Landgrebe

Torben Lubke

Analysis of Lysosomal Structure and Function Using Proteomics and Transcriptomics

130

(1)

Proteome Analysis of Lysosomal Proteins

130

(7)

Transcriptome Analysis of Lysosome Functions

137

(7)

External Lysosomes: The Osteoclast and Its Unique Capacities to Degrade Mineralised Tissues

144

(12)

Vincent Everts

Wouter Beertsen

The Osteoclast as a Highly Polarized Proton-Secreting Cell

144

(12)

Membrane Resealing Mediated by Lysosomal Exocytosis

156

(10)

Norma W. Andrews

``Secretory'' Lysosomes

156

(2)

Ca2+-Regulated Secretion of Conventional Lysosomes

158

(1)

Regulation of Lysosomal Exocytosis by Synaptotagmin VII

158

(8)

Macroautophagy in Mammalian Cells

166

(15)

Eeva-Liisa Eskelinen

Macroautophagic Pathway

166

(5)

Induction and Regulation of Macroautophagy

171

(1)

Functions of Autophagy

172

(1)

Autophagy Genes

173

(2)

Quantitation of Autophagy

175

(2)

Guidelines for Identification of Autophagic Vacuoles in Transmission Electron Microscopy

177

(1)

Future of Autophagy

177

(4)

Chaperone-Mediated Autophagy

181

(14)

Erwin Knecht

Natalia Salvador

Distinctive Characteristics of Chaperone-Mediated Autophagy

184

(1)

Lysosomes Can Take Up Selectively Ribonuclease A and Other Proteins from Cytosol

185

(1)

An Amino Acid Sequence of Ribonuclease A Is Essential for Its Selective Lysosomal Targeting

185

(2)

The Selective Lysosomal Pathway for the Degradation of Proteins Containing KFERQ-Like Sequences Is Active in Various Cell Types under Certain Conditions

187

(1)

Molecular Chaperones Participate in the Selective Lysosomal Transport of Proteins

187

(2)

LAMP2a Is a Receptor Protein in the Lysosomal Membrane for the Entry of Proteins from the Cytosol into Lysosomes by Chaperone-Mediated Autophagy

189

(6)

Index

195