Protein Misassembly

The role that primary amino acid sequences plays in influencing the partitioning of polypeptides between productive folding and irreversible aggregation pathways has introduced a whole new dimension to the folding problem. The volume deals with the structures of the products of protein misassembly and the role of amino acid sequences in favoring these structures.

PREFACE

ix

(4)

CHRISTIAN B. ANFINSEN

xiii

Protein Misassembly in Vitro

1

(60)

RAINER JAENICKE

ROBERT SECKLER

I. Introduction

1

(1)

II. Protein Denaturation and Aggregation

2

(21)

III. Aggregation of Folding Intermediates

23

(5)

IV. Kinetic Partitioning between Folding and Aggregation

28

(6)

V. Protein Misassembly and Molecular Chaperones

34

(7)

VI. Suppressing Protein Misassembly during Protein Folding in Vitro

41

(11)

VII. Concluding Remarks

52

(1)

References

53

(8)

Oligomer Formation by 3D Domain Swapping: A Model for Protein Assembly and Misassembly

61

(62)

MICHAEL P. SCHLUNEGGER

MELANIE J. BENNETT

DAVID EISENBERG

I. Introduction

61

(2)

II. Definitions of 3D Domain Swapping

63

(2)

III. Examples of 3D Domain Swapping

65

(29)

IV. Energetics of 3D Domain Swapping

94

(4)

V. Roles of Amino Acid Sequence and Three-Dimensional Structure in 3D Domain Swapping

98

(5)

VI. Effects of Protein Environment on 3D Domain Swapping

103

(3)

VII. Protein Aggregation and 3D Domain Swapping: Can an Evolved Interface Lead to Pathological Function?

106

(9)

References

115

(8)

The Structure of Amyloid Fibrils by Electron Microscopy and X-Ray Diffraction

123

(38)

MARGARET SUNDE

COLIN BLAKE

I. Introduction to the Structural Characterization of Amyloid

123

(4)

II. The Structural Hierarchy: Fibrils, Protofilaments, and Subprotofilaments

127

(2)

III. The Development of the Cross-BETA-Model for Amyloid Structure

129

(2)

IV. X-Ray Diffraction Studies of Amyloid Fibrils

131

(9)

V. A Common Core Structure for Amyloid Fibrils

140

(4)

VI. X-Ray Studies of Amyloidogenic Proteins

144

(9)

VII. Biological Implications of the Structural Studies

153

(2)

References

155

(6)

Transthyretin Quaternary and Tertiary Structural Changes Facilitate Misassembly into Amyloid

161

(22)

JEFFERY W. KELLY

WILFREDO COLON

ZHIHONG LAI

HILAL A. LASHUEL

JENNIFER MCCULLOCH

SANDRA L. McCUTCHEN

GRETA J. MIROY

SCOTT A. PETERSON

I. Background and Introduction

161

(6)

II. Understanding the Mechanism of Transthyretin Amyloid Fibril Formation in Vitro under Simulated Lysosomal Conditions

167

(8)

III. Understanding the Mechanistic Basis by Which the FAP Mutations Function to Cause Early Onset Amyloid Disease

175

(3)

IV. Therapeutic Strategies for Intervention in TTR Amyloid Disease

178

(1)

V. Summary

178

(1)

References

179

(4)

Domain Stability in Immunoglobulin Light Chain Deposition Disorders

183

(60)

RONALD WETZEL

I. Introduction

183

(1)

II. Possible Factors in Protein Deposition in Vivo

184

(9)

III. The Structural Basis of V(1) Domain Folding Stability

193

(12)

IV. Light Chain Amyloidosis

205

(18)

V. Other Light Chain Aggregation Disorders

223

(7)

VI. Bacterial Inclusion Body Formation of Light Chain V(L) Domains

230

(5)

VII. Conclusion

235

(1)

References

236

(7)

Mutational Effects on Inclusion Body Formation

243

(22)

SCOTT BETTS

CAMERON HAASE-PETTINGELL

JONATHAN KING

I. Introduction

243

(1)

II. Properties of Inclusion Bodies

244

(3)

III. Protein Misfolding and Disease

247

(1)

IV. Product Loss in Industry, Protein Loss in Biomedical Research

247

(1)

V. Sequence Effects on Protein Aggregation in Vitro

248

(1)

VI. Inclusion Body Precursors and Pathways: Misassembly and Aggregation in Vivo

249

(1)

VII. Mutations That Affect Inclusion Body Formation

250

(9)

VIII. Aggregation versus Oligomerization

259

(1)

IX. Inclusion Body Formation, Chaperonin Function, and Novel Therapies

260

(1)

References

261

(4)

AUTHOR INDEX

265

(10)

SUBJECT INDEX

275

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