Research on dyneins has a direct impact on human diseases, such as viruses and cancer. With an accompanying website showing over 100 streaming videos of cell dynamic behavior for best comprehension of material, Dynein: Structure, Biology and Disease is the only reference covering the structure, biology and application of dynein research to human disease. From bench to bedside, Dynein: Structure, Biology and Disease offers research on fundamental cellular processes to researchers and clinicians across developmental biology, cell biology, molecular biology, biophysics, biomedicine, genetics and medicine.
1. 1.1. Introduction 2. 1.2. Research at Harvard 3. 1.3. Research at the University of Hawaii 4. 1.4. Semi-Retirement in Berkeley 5. 1.5. The Pluses of Working on a Minus-End-Directed Motor 6. 1.6. Epilogue 7. 2. Evolutionary Biology of Dyneins 8. 2.1. Introduction 9. 2.2. Dynein Classification 10. 2.3. Dynein Evolution in Eukaryotes 11. 2.4. Evolution in the Proto-Eukaryote 12. 2.5. The Origins of Dynein 13. 2.6. Summary 14. 3. The AAA+ Powerhouse – Trying to Understand How it Works 15. 3.1. Introduction 16. 3.2. Sequence analysis of the Dynein AAA+ Domains 17. 3.3. Nucleotide Binding in the Motor Domain 18. 3.4. How are the AAA+ Modules Spatially Arranged? 19. 3.5. Communication of the Motor Domain with the Microtubule-Binding Domain 20. 3.6. Transduction of Local Conformational Changes into Motion 21. 3.7. Conclusion 22. 4. Dynein Motor Mechanisms 23. 4.1. The Dynein Engine Room 24. 4.2. The Linker Arm and Powerstroke 25. 4.3. Microtubule Affinity: Binding at A Distance 26. 4.4. A Three-Part Harmony in A Big Block V-8 27. 5. Structural Analysis of Dynein Intermediate and Light Chains 28. 5.1. Introduction 29. 5.2. Abbreviated Background of Light Chains 30. 5.3. Structure of The Apo Light Chains 31. 5.4. Structure of Liganded Light Chains 32. 5.5. LC8 and Tctex1 Promiscuity 33. 5.6. Light Chain Isoforms 34. 5.7. Mammalian Dynein Intermediate Chains 35. 5.8. Molecular Model of the Light Chain–Intermediate Chain Structure 36. 5.9. Light Chains and Cargo 37. 5.10. Post-Translational Modifications 38. 5.11. The Roles of LC8 and Tctex1 on Dynein 39. 5.12. Summary 40. 6. Biophysics of Dynein In Vivo 41. 6.1. Single-Molecule Properties of Dynein In Vitro 42. 6.2. Multiple-Motor Properties of Dynein In Vitro 43. 6.3. Regulation of Dynein In Vitro 44. 6.4. Regulation of Dynein In Vivo 45. 6.5. Single-Molecule Properties of Dynein In Vivo 46. 6.6. Regulation of Unidirectional Dynein-Based Transport: Vesicular Cargos 47. 6.7. Regulation of Unidirectional Dynein-Based Transport: Large Cargos 48. 6.8. Regulation of Bidirectional Dynein-Based Transport 49. 7. Composition and Assembly of Axonemal Dyneins 50. 7.1. Introduction 51. 7.2. Classes of Dynein Components 52. 7.3. Monomeric Inner Dynein Arms 53. 7.4. Dimeric Inner Dynein Arm I1/f 54. 7.5. Outer Dynein Arms 55. 7.6. Inter-Dynein Linkers 56. 7.7. Properties and Organization of Axonemal Dynein Motor Units 57. 7.8. Core WD-Repeat Intermediate Chains Associated with Oligomeric Motors 58. 7.9. Additional Intermediate Chains 59. 7.10. Core Light Chains Associated with Oligomeric Motors 60. 7.11. Regulatory Components 61. 7.12. Docking Motors onto the Axoneme 62. 7.13. Other Dynein-Associated Components 63. 7.14. Pathways of Axonemal Dynein Assembly and Transport 64. 7.15. Conclusions 65. 8. Organization of Dyneins in the Axoneme 66. 8.1. Introduction 67. 8.2. The History of Methodological Development in Structural Research into Axonemal Dynein 68. 8.3. Dynein Arm Arrangement In Situ 69. 8.4. Inner Dynein Arm Structure In Situ 70. 8.5. Outer Dynein Arm Structure In Situ 71. 8.6. Heterogeneity and Asymmetry of Structure and Arrangement of Dynein in the Axoneme 72. 8.7. Thoughts on Dynein Functions Based on In Situ Structure 73. 8.8. Outlook 74. 9. Genetic Approaches to Axonemal Dynein Function in Chlamydomonas and Other Organisms 75. 9.1. Introduction 76. 9.2. Genetic Studies of Chlamydomonas Axonemal Dyneins 77. 9.3. Genetic Studies in Various Organisms 78. 9.4. Conclusion and Perspective 79. 10. Regulation of Axonemal Outer-Arm Dyneins in Cilia 80. 10.1. Introduction: Structure, Subunit Composition, and Arrangement of Outer-Arm Dynein 81. 10.2. Fundamental Sliding Activity of Outer-Arm Dynein 82. 10.3. Intra-Outer-Arm Dynein (Inter-Heavy Chain) Regulations 83. 10.4. Inter-Outer-Arm Dynein Regulation 84. 10.5. Regulation of Outer-Arm Dynein Activity by External Signals 85. 10.6. Conclusion 86. 11. Control of Axonemal Inner Dynein Arms 87. 11.1. Overview 88. 11.2. Organization of the Inner Dynein Arms in the Axoneme 89. 11.3. Regulation of I1 Dynein by Second Messengers and Phosphorylation 90. 11.4. Current Questions 91. 12. Flagellar Motility and the Dynein Regulatory Complex 92. 12.1. Introduction 93. 12.2. The Central Pair, Radial Spokes, and Dynein Regulatory Complex 94. 12.3. The Dynein Heavy Chain Suppressors 95. 12.4. The Dynein Regulatory Complex and the Inner Dynein Arms 96. 12.5. The Dynein Regulatory Complex and Nexin Link 97. 12.6. Localization of Dynein Regulatory Complex Subunits Within the Nexin Link 98. 12.7. Identification and Characterization of Dynein Regulatory Complex and Nexin Subunits 99. 12.8. Function of the Dynein Regulatory Complex–Nexin Link in Motility and Future Directions 100. 13. Regulation of Dynein in Ciliary and Flagellar Movement 101. 13.1. Introduction 102. 13.2. Basic Features of the Components of Cilia and Flagella 103. 13.3. Regulation of Microtubule Sliding in the Axoneme 104. 13.4. Sliding Microtubule Theory and Bend Formation 105. 13.5. The Mechanism of Oscillation 106. 13.6. Outlook 107. 14. Dynein and Intraflagellar Transport 108. 14.1. Introduction 109. 14.2. Intraflagellar Transport 110. 14.3. Discovery of the Cytoplasmic Dynein 2 Heavy Chain and Early Proposals for its Function 111. 14.4. Identification of Cytoplasmic Dynein 2 as The Intraflagellar Transport Retrograde Motor 112. 14.5. Structure and Subunit Content of Cytoplasmic Dynein 2 113. 14.6. Function of Cytoplasmic Dynein 2 and Retrograde Intraflagellar Transport 114. 14.7. Conclusion 115. 15. Cytoplasmic Dynein Function Defined by Subunit Composition 116. 15.1. Introduction 117. 15.2. Heavy Chain (DYNC1H) 118. 15.3. Light Intermediate Chain (DYNC1LI) 119. 15.4. Intermediate Chain (DYNC1I) 120. 15.5. Dynll (LC8 Light Chain) 121. 15.6. DYNLT (Tctex1 Light Chain) 122. 15.7. DYNLRB (Roadblock Light Chain) 123. 15.8. Conclusion 124. 16. Studies of Lissencephaly and Neurodegenerative Disease Reveal Novel Aspects of Cytoplasmic Dynein Regulation 125. 16.1. Introduction 126. 16.2. Extramolecular Regulation of Cytoplasmic Dynein Force Generation by LIS1 and NudE/NudEL 127. 16.3. Intramolecular Regulation of Dynein Processivity and Implications for Neurodegeneration 128. 16.4. Conclusion 129. 17. Insights into Cytoplasmic Dynein Function and Regulation from Fungal Genetics 130. 17.1. Introduction 131. 17.2. Discoveries of Dynein Function in Spindle Orientation/Nuclear Migration 132. 17.3. Identification of Dynein Regulators Using Fungal Genetics 133. 17.4. Dissecting the Mechanism and Function of the Microtubule-Plus-End Accumulation of Cytoplasmic Dynein 134. 17.5. Understanding The Functions of Various Components of the Dynein and Dynactin Complexes 135. 17.6. Conclusions 136. 18. Genetic Insights into Mammalian Cytoplasmic Dynein Function Provided by Novel Mutations in the Mouse 137. 18.1. Why, and How, We Work with Mice to Learn About Mammalian Genes such as the Dynein Subunits 138. 18.2. Approaches to Working with Mouse Mutants to Learn About Dynein Function 139. 18.3. Accessing Information About Mouse Mutants 140. 18.4. Genetic Background is Important 141. 18.5. Mouse Strains with Mutations in Cytoplasmic Dynein Subunits 142. 18.6. A Further Note on Nomenclature 143. 18.7. An Allelic Series of Mutations in the Mouse Cytoplasmic Dynein Heavy Chain Gene, Dync1h1 144. 18.8. Overall Conclusions from the Dync1h1 Mutant Mice 145. 18.9. The MMTV-DLCS88A Transgenic Mouse 146. 18.10. Using Mouse Genetics to Further Unravel the Role of the Cytoplasmic Dynein Heavy Chain 147. 18.11. Conclusion 148. 19. The Role of Dynactin in Dynein-Mediated Motility 149. 19.1. Introduction 150. 19.2. Structure and Composition of Dynactin 151. 19.3. Known Activities of Dynactin 152. 19.4. Dynactin Function in Dynein-Based Motility 153. 20. Roles of Cytoplasmic Dynein During Mitosis 154. 20.1. Introduction 155. 20.2. Model Systems of Mitotic Dynein 156. 20.3. Spindle Pole Dynein 157. 20.4. Cortical Dynein 158. 20.5. Kinetochore Dynein 159. 20.6. Phosphorylation 160. 20.7. Future Questions 161. 20.8. Conclusions 162. 21. Does Dynein Influence the Non-Mendelian Inheritance of Chromosome 17 Homologs in Male Mice? 163. 21.1. Introduction 164. 21.2. Properties of Tctex1 165. 21.3. Properties of Tctex2 166. 21.4. Properties of Dnahc8 167. 21.5. Chromosomal Deletion Analysis Modifies Lyon’s Model 168. 21.6. Identification of Tcds 169. 21.7. What About Dyneins? 170. 22. Role of Dynein in Viral Pathogenesis 171. 22.1. General Tenets of Virus–Host Interactions 172. 22.2. Blocking Dynein Function in Virus-Infected Cells: Potential Drug Development to Poison Viral Replication Steps 173. 22.3. Direct Interactions with Dynein for Viral Entry: Trafficking Towards the Nucleus by Various Viruses 174. 22.4. Innate Immune Response to Viral Infection 175. 22.5. Dyneins and Stress Granule Assembly: A Novel Concept in Innate Responses to Viral Infection 176. 22.6. Dynein Involvement in Viral Egress and Assembly 177. 22.7. Using Dynein To Traffic to Virus Assembly Domains 178. 22.8. Cell-to-Cell Transmission 179. 22.9. Virus Export from Virus Factories 180. 22.10. Concluding Remarks 181. 23. Cytoplasmic Dynein Dysfunction and Neurodegenerative Disease 182. 23.1. Introduction 183. 23.2. Cytoplasmic Dynein Drives Intracellular Transport in Neurons 184. 23.3. Dynein Function in Developing Neurons 185. 23.4. Dynein Dysfunction in Neurodegeneration 186. 23.5. Dynein Mutations in Model Organisms 187. 23.6. Dynein's Role in the Pathogenesis of Common Neurodegenerative Disease 188. 23.7. Conclusions 189. 24. Dynein Dysfunction as a Cause of Primary Ciliary Dyskinesia and Other Ciliopathies 190. 24.1. Introduction 191. 24.2. Ultrastructure of Motile Cilia 192. 24.3. Outer Dynein Arms 193. 24.4. Inner Dynein Arms 194. 24.5. Ciliopathies 195. 24.6. Primary Cilia Dyskinesia 196. 24.7. Molecular Defects Affecting Outer Dynein Arm Components 197. 24.8. Molecular Defects Affecting Outer and Inner Dynein Arms 198. 24.9. Molecular Defects Affecting the Central Pair and Radial Spokes 199. 24.10. Molecular Defects Affecting Dynein Regulatory Complexes and Inner Dynein Arms 200. Index
Research on dyneins has a direct impact on human diseases, such as viruses and cancer. With an accompanying website showing over 100 streaming videos of cell dynamic behavior for best comprehension of material, Dynein: Structure, Biology and Disease is the only reference covering the structure, biology and application of dynein research to human disease. From bench to bedside, Dynein: Structure, Biology and Disease offers research on fundamental cellular processes to researchers and clinicians across developmental biology, cell biology, molecular biology, biophysics, biomedicine, genetics and medicine.
1. 1.1. Introduction 2. 1.2. Research at Harvard 3. 1.3. Research at the University of Hawaii 4. 1.4. Semi-Retirement in Berkeley 5. 1.5. The Pluses of Working on a Minus-End-Directed Motor 6. 1.6. Epilogue 7. 2. Evolutionary Biology of Dyneins 8. 2.1. Introduction 9. 2.2. Dynein Classification 10. 2.3. Dynein Evolution in Eukaryotes 11. 2.4. Evolution in the Proto-Eukaryote 12. 2.5. The Origins of Dynein 13. 2.6. Summary 14. 3. The AAA+ Powerhouse – Trying to Understand How it Works 15. 3.1. Introduction 16. 3.2. Sequence analysis of the Dynein AAA+ Domains 17. 3.3. Nucleotide Binding in the Motor Domain 18. 3.4. How are the AAA+ Modules Spatially Arranged? 19. 3.5. Communication of the Motor Domain with the Microtubule-Binding Domain 20. 3.6. Transduction of Local Conformational Changes into Motion 21. 3.7. Conclusion 22. 4. Dynein Motor Mechanisms 23. 4.1. The Dynein Engine Room 24. 4.2. The Linker Arm and Powerstroke 25. 4.3. Microtubule Affinity: Binding at A Distance 26. 4.4. A Three-Part Harmony in A Big Block V-8 27. 5. Structural Analysis of Dynein Intermediate and Light Chains 28. 5.1. Introduction 29. 5.2. Abbreviated Background of Light Chains 30. 5.3. Structure of The Apo Light Chains 31. 5.4. Structure of Liganded Light Chains 32. 5.5. LC8 and Tctex1 Promiscuity 33. 5.6. Light Chain Isoforms 34. 5.7. Mammalian Dynein Intermediate Chains 35. 5.8. Molecular Model of the Light Chain–Intermediate Chain Structure 36. 5.9. Light Chains and Cargo 37. 5.10. Post-Translational Modifications 38. 5.11. The Roles of LC8 and Tctex1 on Dynein 39. 5.12. Summary 40. 6. Biophysics of Dynein In Vivo 41. 6.1. Single-Molecule Properties of Dynein In Vitro 42. 6.2. Multiple-Motor Properties of Dynein In Vitro 43. 6.3. Regulation of Dynein In Vitro 44. 6.4. Regulation of Dynein In Vivo 45. 6.5. Single-Molecule Properties of Dynein In Vivo 46. 6.6. Regulation of Unidirectional Dynein-Based Transport: Vesicular Cargos 47. 6.7. Regulation of Unidirectional Dynein-Based Transport: Large Cargos 48. 6.8. Regulation of Bidirectional Dynein-Based Transport 49. 7. Composition and Assembly of Axonemal Dyneins 50. 7.1. Introduction 51. 7.2. Classes of Dynein Components 52. 7.3. Monomeric Inner Dynein Arms 53. 7.4. Dimeric Inner Dynein Arm I1/f 54. 7.5. Outer Dynein Arms 55. 7.6. Inter-Dynein Linkers 56. 7.7. Properties and Organization of Axonemal Dynein Motor Units 57. 7.8. Core WD-Repeat Intermediate Chains Associated with Oligomeric Motors 58. 7.9. Additional Intermediate Chains 59. 7.10. Core Light Chains Associated with Oligomeric Motors 60. 7.11. Regulatory Components 61. 7.12. Docking Motors onto the Axoneme 62. 7.13. Other Dynein-Associated Components 63. 7.14. Pathways of Axonemal Dynein Assembly and Transport 64. 7.15. Conclusions 65. 8. Organization of Dyneins in the Axoneme 66. 8.1. Introduction 67. 8.2. The History of Methodological Development in Structural Research into Axonemal Dynein 68. 8.3. Dynein Arm Arrangement In Situ 69. 8.4. Inner Dynein Arm Structure In Situ 70. 8.5. Outer Dynein Arm Structure In Situ 71. 8.6. Heterogeneity and Asymmetry of Structure and Arrangement of Dynein in the Axoneme 72. 8.7. Thoughts on Dynein Functions Based on In Situ Structure 73. 8.8. Outlook 74. 9. Genetic Approaches to Axonemal Dynein Function in Chlamydomonas and Other Organisms 75. 9.1. Introduction 76. 9.2. Genetic Studies of Chlamydomonas Axonemal Dyneins 77. 9.3. Genetic Studies in Various Organisms 78. 9.4. Conclusion and Perspective 79. 10. Regulation of Axonemal Outer-Arm Dyneins in Cilia 80. 10.1. Introduction: Structure, Subunit Composition, and Arrangement of Outer-Arm Dynein 81. 10.2. Fundamental Sliding Activity of Outer-Arm Dynein 82. 10.3. Intra-Outer-Arm Dynein (Inter-Heavy Chain) Regulations 83. 10.4. Inter-Outer-Arm Dynein Regulation 84. 10.5. Regulation of Outer-Arm Dynein Activity by External Signals 85. 10.6. Conclusion 86. 11. Control of Axonemal Inner Dynein Arms 87. 11.1. Overview 88. 11.2. Organization of the Inner Dynein Arms in the Axoneme 89. 11.3. Regulation of I1 Dynein by Second Messengers and Phosphorylation 90. 11.4. Current Questions 91. 12. Flagellar Motility and the Dynein Regulatory Complex 92. 12.1. Introduction 93. 12.2. The Central Pair, Radial Spokes, and Dynein Regulatory Complex 94. 12.3. The Dynein Heavy Chain Suppressors 95. 12.4. The Dynein Regulatory Complex and the Inner Dynein Arms 96. 12.5. The Dynein Regulatory Complex and Nexin Link 97. 12.6. Localization of Dynein Regulatory Complex Subunits Within the Nexin Link 98. 12.7. Identification and Characterization of Dynein Regulatory Complex and Nexin Subunits 99. 12.8. Function of the Dynein Regulatory Complex–Nexin Link in Motility and Future Directions 100. 13. Regulation of Dynein in Ciliary and Flagellar Movement 101. 13.1. Introduction 102. 13.2. Basic Features of the Components of Cilia and Flagella 103. 13.3. Regulation of Microtubule Sliding in the Axoneme 104. 13.4. Sliding Microtubule Theory and Bend Formation 105. 13.5. The Mechanism of Oscillation 106. 13.6. Outlook 107. 14. Dynein and Intraflagellar Transport 108. 14.1. Introduction 109. 14.2. Intraflagellar Transport 110. 14.3. Discovery of the Cytoplasmic Dynein 2 Heavy Chain and Early Proposals for its Function 111. 14.4. Identification of Cytoplasmic Dynein 2 as The Intraflagellar Transport Retrograde Motor 112. 14.5. Structure and Subunit Content of Cytoplasmic Dynein 2 113. 14.6. Function of Cytoplasmic Dynein 2 and Retrograde Intraflagellar Transport 114. 14.7. Conclusion 115. 15. Cytoplasmic Dynein Function Defined by Subunit Composition 116. 15.1. Introduction 117. 15.2. Heavy Chain (DYNC1H) 118. 15.3. Light Intermediate Chain (DYNC1LI) 119. 15.4. Intermediate Chain (DYNC1I) 120. 15.5. Dynll (LC8 Light Chain) 121. 15.6. DYNLT (Tctex1 Light Chain) 122. 15.7. DYNLRB (Roadblock Light Chain) 123. 15.8. Conclusion 124. 16. Studies of Lissencephaly and Neurodegenerative Disease Reveal Novel Aspects of Cytoplasmic Dynein Regulation 125. 16.1. Introduction 126. 16.2. Extramolecular Regulation of Cytoplasmic Dynein Force Generation by LIS1 and NudE/NudEL 127. 16.3. Intramolecular Regulation of Dynein Processivity and Implications for Neurodegeneration 128. 16.4. Conclusion 129. 17. Insights into Cytoplasmic Dynein Function and Regulation from Fungal Genetics 130. 17.1. Introduction 131. 17.2. Discoveries of Dynein Function in Spindle Orientation/Nuclear Migration 132. 17.3. Identification of Dynein Regulators Using Fungal Genetics 133. 17.4. Dissecting the Mechanism and Function of the Microtubule-Plus-End Accumulation of Cytoplasmic Dynein 134. 17.5. Understanding The Functions of Various Components of the Dynein and Dynactin Complexes 135. 17.6. Conclusions 136. 18. Genetic Insights into Mammalian Cytoplasmic Dynein Function Provided by Novel Mutations in the Mouse 137. 18.1. Why, and How, We Work with Mice to Learn About Mammalian Genes such as the Dynein Subunits 138. 18.2. Approaches to Working with Mouse Mutants to Learn About Dynein Function 139. 18.3. Accessing Information About Mouse Mutants 140. 18.4. Genetic Background is Important 141. 18.5. Mouse Strains with Mutations in Cytoplasmic Dynein Subunits 142. 18.6. A Further Note on Nomenclature 143. 18.7. An Allelic Series of Mutations in the Mouse Cytoplasmic Dynein Heavy Chain Gene, Dync1h1 144. 18.8. Overall Conclusions from the Dync1h1 Mutant Mice 145. 18.9. The MMTV-DLCS88A Transgenic Mouse 146. 18.10. Using Mouse Genetics to Further Unravel the Role of the Cytoplasmic Dynein Heavy Chain 147. 18.11. Conclusion 148. 19. The Role of Dynactin in Dynein-Mediated Motility 149. 19.1. Introduction 150. 19.2. Structure and Composition of Dynactin 151. 19.3. Known Activities of Dynactin 152. 19.4. Dynactin Function in Dynein-Based Motility 153. 20. Roles of Cytoplasmic Dynein During Mitosis 154. 20.1. Introduction 155. 20.2. Model Systems of Mitotic Dynein 156. 20.3. Spindle Pole Dynein 157. 20.4. Cortical Dynein 158. 20.5. Kinetochore Dynein 159. 20.6. Phosphorylation 160. 20.7. Future Questions 161. 20.8. Conclusions 162. 21. Does Dynein Influence the Non-Mendelian Inheritance of Chromosome 17 Homologs in Male Mice? 163. 21.1. Introduction 164. 21.2. Properties of Tctex1 165. 21.3. Properties of Tctex2 166. 21.4. Properties of Dnahc8 167. 21.5. Chromosomal Deletion Analysis Modifies Lyon’s Model 168. 21.6. Identification of Tcds 169. 21.7. What About Dyneins? 170. 22. Role of Dynein in Viral Pathogenesis 171. 22.1. General Tenets of Virus–Host Interactions 172. 22.2. Blocking Dynein Function in Virus-Infected Cells: Potential Drug Development to Poison Viral Replication Steps 173. 22.3. Direct Interactions with Dynein for Viral Entry: Trafficking Towards the Nucleus by Various Viruses 174. 22.4. Innate Immune Response to Viral Infection 175. 22.5. Dyneins and Stress Granule Assembly: A Novel Concept in Innate Responses to Viral Infection 176. 22.6. Dynein Involvement in Viral Egress and Assembly 177. 22.7. Using Dynein To Traffic to Virus Assembly Domains 178. 22.8. Cell-to-Cell Transmission 179. 22.9. Virus Export from Virus Factories 180. 22.10. Concluding Remarks 181. 23. Cytoplasmic Dynein Dysfunction and Neurodegenerative Disease 182. 23.1. Introduction 183. 23.2. Cytoplasmic Dynein Drives Intracellular Transport in Neurons 184. 23.3. Dynein Function in Developing Neurons 185. 23.4. Dynein Dysfunction in Neurodegeneration 186. 23.5. Dynein Mutations in Model Organisms 187. 23.6. Dynein's Role in the Pathogenesis of Common Neurodegenerative Disease 188. 23.7. Conclusions 189. 24. Dynein Dysfunction as a Cause of Primary Ciliary Dyskinesia and Other Ciliopathies 190. 24.1. Introduction 191. 24.2. Ultrastructure of Motile Cilia 192. 24.3. Outer Dynein Arms 193. 24.4. Inner Dynein Arms 194. 24.5. Ciliopathies 195. 24.6. Primary Cilia Dyskinesia 196. 24.7. Molecular Defects Affecting Outer Dynein Arm Components 197. 24.8. Molecular Defects Affecting Outer and Inner Dynein Arms 198. 24.9. Molecular Defects Affecting the Central Pair and Radial Spokes 199. 24.10. Molecular Defects Affecting Dynein Regulatory Complexes and Inner Dynein Arms 200. Index