Front Cover; Prion Protein; Copyright; Contents; Contributors; Preface; Chapter One: Functions of the Prion Protein; 1. Introduction; 2. The Basics of PrPC; 2.1. PrPC From Gene to Function; 2.2. PrPC Expression and Localization; 3. Lessons From Knockout Models; 4. Miscellaneous Roles in CNS Function; 4.1. Cognitive and Behavioral Functions; 4.2. Neuritogenesis and Neuronal Differentiation; 4.3. Balancing Neuroprotection and Neurotoxicity; 4.4. Beyond Neurons: PrPC in Glia; 4.5. The Metal Connection; 4.6. From Neurotransmitter Receptors to Neurotransmitter-Associated Functions

5. Cell Fate and Differentiation5.1. Stem Cell Self-renewal and Expansion; 5.2. A Hub in Developmental Signaling Pathways; 5.3. Choice of Cell Fate and Differentiation; 6. A Broad Protective Role Against Stress; 6.1. PrPC Is Induced Under Stress; 6.2. Protection Against Oxidative Stress; 6.3. Protection Against Genotoxic Stress; 6.4. Protection Against Apoptosis; 6.5. An Immune Privilege Molecule?; 6.6. Involvement in Proteostasis; 7. Cell Adhesion and ECM Contacts; 7.1. Multiple Adhesion Partners; 7.2. Special Links With Cadherins; 7.3. Modulating the ECM Composition

8. From Partners to Cell Signaling8.1. Cell Signaling: The Unifying Function of PrPC?; 8.2. A Great Variety of Partners; 8.3. Overview of PrPC-Dependent Signal Transduction; 9. Concluding Remarks; References; Chapter Two: Copper- and Zinc-Promoted Interdomain Structure in the Prion Protein: A Mechanism for Autoinhibition of the ... ; 1. Introduction; 1.1. PrPC Structure; 1.2. Molecular Features of Cu2+ and Zn2+ Binding; 1.3. Metal Ions and PrPC Function; 2. The N-Terminal Toxicity Hypothesis; 2.1. Internal Deletions of PrPC; 2.2. N-Terminal Toxicity Induced by GD Ligands

3. Cu2+ and Zn2+ Promote Interdomain Interaction in cis4. Disruption of cis Interaction as a Mechanism of Neurotoxicity; 5. Concluding Remarks; Acknowledgment; References; Chapter Three: Cell Biology of Prion Protein; 1. Expression and Function of Prion Protein; 2. PrPC: The Principal Actor in Prion Replication; 3. Structure and Biosynthesis of PrPC; 4. Trafficking of Cellular Prion Protein; 5. Several Topological Forms of PrPC; 6. Quality Control of Cellular Prion Protein; 7. GPI-Anchor: Structure and Biosynthesis; 8. Functions of GPI-Anchored Proteins; 9. GPI-Anchor Function

10. GPI-Anchor and PrionReferences; Chapter Four: Understanding the Effect of Disease-Related Mutations on Human Prion Protein Structure: Insights From NMR S ... ; 1. Introduction; 2. Structural Features of PrPC and PrPSc; 3. Strategy for Resonance Assignment and NMR Structure Determination of Human Prion Protein Variants; 4. Structural Features of Human Prion Proteins With Pathological Mutations; 4.1. Mildly Acidic pH Conditions; 4.2. Physiological pH Conditions; 5. Structural Features of Human Prion Protein With Protective E219K Polymorphism; 6. Concluding Remarks; Acknowledgment; References