Akio Koizumi, Department of Health and Environmental Sciences, Graduate School of Medicine, Kyoto UniversityKazuhiro Nagata, Laboratory of Molecular and Cellular biology, Faculty of Life Sciences, Kyoto Sangyo UniversityKiyohiro Houkin, Department of Neurosurgery, Graduate School of Medicine, Hokkaido UniversityTeiji Tominaga, Department of Neurosurgery, Tohoku University School of Medicine Susumu Miyamoto, Department of Neurosurgery, Graduate School of Medicine, Kyoto UniversityShigeo Kure, Department of Pediatrics, Tohoku University School of MedicineElizabeth Tournier‐Lasserve, CHU Paris‐GH St‐Louis Lariboisière F‐Widal ‐ Hôpital Lariboisière

• Part I: Prologue.-1. A prologue to moyamoya disease and RNF213.-2. Concept of moyamoya Disease.-Part II: Genetic Epidemiology.-3. Molecular Epidemiology in East Asian Countries and in the World.-Part III: Biochemistry, Function and Molecular Pathology.-4. Molecular Biology of Mysterin/RNF213.-5. Physiological Role of Mysterin/RNF213 in Zebrafish.-6. Pathological Investigation on RNF213: Animal Models Knockout and Transgenic Mice in Diabetes and Signal Transduction.-7. Pathological Investigation on RNF213: Animal Models of Rnf213-knockout and Knock-in Mice.-8. RNF213 and Circulating Factors in Patients with Moyamoya Disease .-9. Genetics of Moyamoya beyond RNF213.  Monogenic Moyamoya Syndromes.-Part IV: Clinical Management10. Natural History and Long-term Clinical Outcome after Conservative and Surgical Management.-11. Significance of RNF213 in Clinical Management in Japan.-12. Significance of RNF213 in Clinical Management in China.-13. The Different Forms of Moyamoya Disease and Their Clinical Management.-14. Vascular Diseases Attributable to RNF213 Other than Moyamoya Disease.-Part V: Epilogue.-Future Clinical Perspectives on RNF213 in Moyamoya Disease.

Akio Koizumi, Department of Health and Environmental Sciences, Graduate School of Medicine, Kyoto UniversityKazuhiro Nagata, Laboratory of Molecular and Cellular biology, Faculty of Life Sciences, Kyoto Sangyo UniversityKiyohiro Houkin, Department of Neurosurgery, Graduate School of Medicine, Hokkaido UniversityTeiji Tominaga, Department of Neurosurgery, Tohoku University School of Medicine Susumu Miyamoto, Department of Neurosurgery, Graduate School of Medicine, Kyoto UniversityShigeo Kure, Department of Pediatrics, Tohoku University School of MedicineElizabeth Tournier‐Lasserve, CHU Paris‐GH St‐Louis Lariboisière F‐Widal ‐ Hôpital Lariboisière

• Part I: Prologue.-1. A prologue to moyamoya disease and RNF213.-2. Concept of moyamoya Disease.-Part II: Genetic Epidemiology.-3. Molecular Epidemiology in East Asian Countries and in the World.-Part III: Biochemistry, Function and Molecular Pathology.-4. Molecular Biology of Mysterin/RNF213.-5. Physiological Role of Mysterin/RNF213 in Zebrafish.-6. Pathological Investigation on RNF213: Animal Models Knockout and Transgenic Mice in Diabetes and Signal Transduction.-7. Pathological Investigation on RNF213: Animal Models of Rnf213-knockout and Knock-in Mice.-8. RNF213 and Circulating Factors in Patients with Moyamoya Disease .-9. Genetics of Moyamoya beyond RNF213.  Monogenic Moyamoya Syndromes.-Part IV: Clinical Management10. Natural History and Long-term Clinical Outcome after Conservative and Surgical Management.-11. Significance of RNF213 in Clinical Management in Japan.-12. Significance of RNF213 in Clinical Management in China.-13. The Different Forms of Moyamoya Disease and Their Clinical Management.-14. Vascular Diseases Attributable to RNF213 Other than Moyamoya Disease.-Part V: Epilogue.-Future Clinical Perspectives on RNF213 in Moyamoya Disease.

Akio Koizumi, Department of Health and Environmental Sciences, Graduate School of Medicine, Kyoto UniversityKazuhiro Nagata, Laboratory of Molecular and Cellular biology, Faculty of Life Sciences, Kyoto Sangyo UniversityKiyohiro Houkin, Department of Neurosurgery, Graduate School of Medicine, Hokkaido UniversityTeiji Tominaga, Department of Neurosurgery, Tohoku University School of Medicine Susumu Miyamoto, Department of Neurosurgery, Graduate School of Medicine, Kyoto UniversityShigeo Kure, Department of Pediatrics, Tohoku University School of MedicineElizabeth Tournier‐Lasserve, CHU Paris‐GH St‐Louis Lariboisière F‐Widal ‐ Hôpital Lariboisière

• Part I: Prologue.-1. A prologue to moyamoya disease and RNF213.-2. Concept of moyamoya Disease.-Part II: Genetic Epidemiology.-3. Molecular Epidemiology in East Asian Countries and in the World.-Part III: Biochemistry, Function and Molecular Pathology.-4. Molecular Biology of Mysterin/RNF213.-5. Physiological Role of Mysterin/RNF213 in Zebrafish.-6. Pathological Investigation on RNF213: Animal Models Knockout and Transgenic Mice in Diabetes and Signal Transduction.-7. Pathological Investigation on RNF213: Animal Models of Rnf213-knockout and Knock-in Mice.-8. RNF213 and Circulating Factors in Patients with Moyamoya Disease .-9. Genetics of Moyamoya beyond RNF213.  Monogenic Moyamoya Syndromes.-Part IV: Clinical Management10. Natural History and Long-term Clinical Outcome after Conservative and Surgical Management.-11. Significance of RNF213 in Clinical Management in Japan.-12. Significance of RNF213 in Clinical Management in China.-13. The Different Forms of Moyamoya Disease and Their Clinical Management.-14. Vascular Diseases Attributable to RNF213 Other than Moyamoya Disease.-Part V: Epilogue.-Future Clinical Perspectives on RNF213 in Moyamoya Disease.