Analysis of pathophysiology of hereditary cerebral amyloid angiopathy associated with amyloidgenic transthyretin Y114C
Project/Area Number |
23500430
|
Research Category |
Grant-in-Aid for Scientific Research (C)
|
Allocation Type | Multi-year Fund |
Section | 一般 |
Research Field |
Nerve anatomy/Neuropathology
|
Research Institution | Kumamoto University |
Principal Investigator |
YAMASHITA Taro 熊本大学, 生命科学研究部, 講師 (90381003)
|
Co-Investigator(Kenkyū-buntansha) |
ANDO Yukio 熊本大学, 生命科学研究部・神経内科学分野, 教授 (20253742)
HIRAHARA Tomoo 熊本大学, 医学部附属病院・神経内科, 助教 (80649343)
|
Project Period (FY) |
2011 – 2013
|
Project Status |
Completed (Fiscal Year 2013)
|
Budget Amount *help |
¥4,940,000 (Direct Cost: ¥3,800,000、Indirect Cost: ¥1,140,000)
Fiscal Year 2013: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2012: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2011: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
|
Keywords | 脳アミロド血管症 / トランスサイレチン / 認知症 / 脳出血 / 脳血管透過性亢進 / 肝移植 / 治療 / 脳アミロイド血管症 / 血管透過性亢進 / 微小脳出血 / 脳血管炎 / アミロイド血管症 |
Research Abstract |
Patients with amyloidogenic transthyretin (TTR) Y114C develop amyloid deposits in cerebral blood vessels, cerebral hemorrhage, and rapidly progressive dementia that presents with hereditary cerebral amyloid angiopathy (CAA). MRI studies with FLAIR images after Gd administration showed Gd leakage into the subarachnoid space. The pathogenesis of CNS disorders may reflect disruption of the blood-CSF barrier and blood-brain barrier by amyloid deposition. The mortality and occurrence of cerebral hemorrhage and dementia in patients having transplantations were reduced compared with those in patients not having transplantations. The group undergoing transplantations had significantly smaller volumes of intracranial hemorrhage than did the no-transplantation group. Liver transplantation was effective for CNS manifestations of cerebral amyloid angiopathy associated with amyloidogenic transthyretin Y114C.
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Report
(4 results)
Research Products
(58 results)
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[Journal Article] Gait Disturbance due to Foot Drop Is Refractory to Treatment in Nonsystemic Vasculitic Neuropathy2014
Author(s)
Hirahara T, Yamashita S, Misumi Y, Kawakami K, Hori H, Honda S, Watanabe M, Ikeda T, Yamashita T, Maeda Y, Ando Y
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Journal Title
Eur Neurol
Volume: 71
Issue: 3-4
Pages: 180-186
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[Journal Article] Repurposing diflunisal for familial amyloid polyneuropathy: a randomized clinical trial2013
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Berk JL, Suhr OB, Obici L, Sekijima Y, Zeldenrust SR, Yamashita T, Heneghan MA, Gorevic PD, Litchy WJ, Wiesman JF, Nordh E, Corato M, Lozza A, Cortese A, Robinson-Papp J, Colton T, Rybin DV, Bisbee AB, Ando Y, Ikeda S, Seldin DC, Merlini G, Skinner M, Kelly JW, Dyck PJ; Diflunisal Trial Consortium
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Journal Title
JAMA
Volume: 310
Issue: 24
Pages: 2658-2667
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[Journal Article] Effect of age and sex differences on wild-type transthyretin amyloid formation in familial amyloidotic polyneuropathy: a proteomic approach2013
Author(s)
Tasaki M, Ueda M, Obayashi K, Koike H, Kitagawa K, Ogi Y, Jono H, Su Y, Suenaga G, Oshima T, Misumi Y, Yoshida M, Yamashita T, Sobue G, Ando Y
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Journal Title
Int J Cardiol
Volume: 170
Issue: 1
Pages: 69-74
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[Journal Article] Changes in pathological and biochemical findings of systemic tissue sites in familial amyloid polyneuropathy more than 10 years after liver transplantation2013
Author(s)
Oshima T, Kawahara S, Ueda M, Kawakami Y, Tanaka R, Okazaki T, Misumi Y, Obayashi K, Yamashita T, Ohya Y, Ihse E, Shinriki S, Tasaki M, Jono H, Asonuma K, Inomata Y, Westermark P, Ando Y
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J Neurol Neurosurg Psychiatry
Volume: -
Issue: 7
Pages: 740-746
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[Journal Article] Dominant cardiac type of familial amyloidotic polyneuropathy associated with a novel transthyretin variant Thr59Arg2013
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Obayashi K, Misumi Y, Watanabe T, Ando T, Akagami T, Tasaki M, Shinriki S, Ueda M, Yamashita T, Hirotani S, Ando Y
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Journal Title
Muscle Nerve
Volume: 48(5)
Issue: 5
Pages: 839-41
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[Journal Article] Marked cardiomegaly in a patient with familial amyloidotic polyneuropathy after orthotopic liver transplantation: a case study2013
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Sakashita N, Kagawa S, Date R, Ueno T, Nakagawa T, Yamashita T, Obayashi K, Ohshima T, Ueda M, Horiguchi H, Ando Y, Takeya M
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Journal Title
Pathol Int
Volume: 63
Issue: 5
Pages: 260-265
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[Journal Article] Amyloid fibrils containing fragmented ATTR may be the standard fibril composition in ATTR amyloidosis2013
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Ihse E, Rapezzi C, Merlini G, Benson MD, Ando Y, Suhr OB, Ikeda S, Lavatelli F, Obici L, Quarta CC, Leone O, Jono H, Ueda M, Lorenzini M, Liepnieks J, Ohshima T, Tasaki M, Yamashita T, Westermark P
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Journal Title
Amyloid
Volume: 20(3)
Issue: 3
Pages: 142-50
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[Journal Article] Impact of antibodies against amyloidogenic transthyretin (ATTR) on phenotypes of patients with familial amyloidotic polyneuropathy (FAP) ATTR Valine30Methionine2013
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Obayashi K, Tasaki M, Jono H, Ueda M, Shinriki S, Misumi Y, Yamashita T, Oshima T, Nakamura T, Ikemizu S, Anan I, Suhr O, Ando Y
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Journal Title
Clin Chim Acta
Volume: 419
Pages: 127-31
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[Journal Article] Muscle biopsy findings predictive of malignancy in rare infiltrative dermatomyositis2013
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Uchino M, Yamashita S, Uchino K, Mori A, Hara A, Suga T, Hirahara T, Koide T, Kimura E, Yamashita T, Ueda A, Kurisaki R, Suzuki J, Honda S, Maeda Y, Hirano T, Ando Y
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Journal Title
Clin Neurol Neurosurg
Volume: 115(5)
Issue: 5
Pages: 603-6
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[Journal Article] Long-term survival after liver transplantation in patients with familial amyloid polyneuropathy2012
Author(s)
Yamashita T, Ando Y, Okamoto S, Misumi Y, Hirahara T, Ueda M, Obayashi K, Nakamura M, Jono H, Shono M, Asonuma K, Inomata Y, Uchino M
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Journal Title
Neurology
Volume: 78(9)
Issue: 9
Pages: 637-43
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[Journal Article] The Diflunisal Trial: study accrual and drug tolerance.2012
Author(s)
Berk JL, Suhr OB, Sekijima Y, Yamashita T, Heneghan M, Zeldenrust SR, Ando Y, Ikeda S, Gorevic P, Merlini G, Kelly JW, Skinner M, Bisbee AB, Dyck PJ, Obici L
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Journal Title
Amyloid
Volume: Suppl 1
Issue: sup1
Pages: 37-38
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[Journal Article] Long-term survival after liver transplantation in patients with familial amyloid polyneuropathy2012
Author(s)
Yamashita T, Ando Y, Okamoto S, Misumi Y, Hirahara T, Ueda M, Obayashi K, Nakamura M, Jono H, Shono M, Asonuma K, Inomata Y, Uchino M.
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Journal Title
Neurology
Volume: 78
Pages: 637-643
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[Journal Article] Effect of liver transplantation on the survival of patients with ordinary onset familial amyloid polyneuropathy in Japan2011
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Yamashita T, Ando Y, Okamoto S, Yohei M, Hitahara T, Ueda M, Obayashi K, Nakamura M, Jono H, Asonuma K, Inomata Y, Uchino M
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Journal Title
Amyloid
Volume: Suppl1
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Pages: 185-6
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[Journal Article] Spinal multifocal amyloidosis derived from wild-type transthyretin2011
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Sueyoshi T, Ueda M, Sei A, Misumi Y, Oshima T, Yamashita T, Obayashi K, Shinriki S, Jono H, Shono M, Ando Y, Mizuta H
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Journal Title
Amyloid
Volume: 8(3)
Issue: 3
Pages: 165-8
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[Journal Article] Amyloid neuropathy in a younger domino liver transplanted recipient2011
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Obayashi K, Yamashita T, Tasaki M, Ueda M, Shono M, Jono H, Ohshima T, Ohya Y, Asonuma K, Inomata Y, Ando Y
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Journal Title
Muscle Nerve
Volume: 3(3)
Issue: 3
Pages: 449-50
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[Journal Article] Effect of liver transplantation on the survival of patients with ordinary onset familial amyloid polyneuropathy in Japan2011
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Yamashita T, Ando Y, Okamoto S, Yohei M, Hirahara T, Ueda M, Obayashi K, Nakamura M, Jono H, Asonuma K, Inomata Y, Uchino M
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Journal Title
Amyloid
Volume: 18 Suppl 1
Pages: 180-181
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[Journal Article] A homozygote case of familial amyloid polyneuropathy amyloidgenic transthyretin Val30Met in a non-endemic area2011
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Kumabe Y, Miura A, Yamashita T, Oshima T, Misumi Y, Ueda M, Obayashi K, Horibata Y, Jono H, Ando Y, Uchino M
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山下太郎, 阪本徹郎, 栗﨑玲一, 植川和利, 三隅洋平, 植田光晴, 平原智雄, 渡邉聖樹, 山下賢, 前田寧, 大林光念, 安東由喜雄
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Year and Date
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第1回日本アミロイドーシス研究会学術集会
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American Academy of Neurology 65th Annual Meeting
Place of Presentation
San Diego, USA
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[Presentation] FAP患者肝を用いたドミノ移植患者剖検例におけるアミロイド沈着様式の検討2013
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山下太郎, 小池春樹, 大嶋俊範, 磯野香織, 田崎雅義, 三隅洋平, 植田光晴, 神力 悟,大林光念, 城野博史, 祖父江元, 安東由喜雄
Organizer
平成24年度アミロイドーシスに関する調査研究班 研究報告会
Place of Presentation
東京, KKRホテル東京
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XIII International Symposium on Amyloidosis
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Groningen, The Netherlands
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[Presentation] ATTR Y114C 型脳アミロイド血管症におけるアミロイド沈着の分布の解析2012
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山下太郎, 大嶋俊範, 奥村幸祐, 永利聡仁, 立石真知子, 三隅洋平, 神力 悟, 植田光晴, 大林光念, 城野博史, 安東由喜雄
Organizer
第53回日本神経学会学術大会
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奥村幸祐, 山下太郎, 立石真知子, 大嶋俊範, 植田明彦, 三隅洋平, 平原智雄, 本田省ニ, 植田光晴, 大林光念, 山下 賢, 前田 寧, 安東由喜雄
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第30回日本神経治療学会総会
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[Presentation] ATTR Y114C型脳アミロイド血管症におけるアミロイド沈着の分布の解析2012
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Place of Presentation
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[Presentation] Effect of liver transplantation on clinical manifestations of familial amyloid polyneuropathy2011
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Organizer
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Author(s)
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VIIIth International Symposium on Familial Amyloidotic Polyneuropathy and VIIth International Symposium on Liver Transplantation in Familial Amyloidotic Polyneuropathy
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[Presentation] Effect of liver transplantation on clinical manifestations of familial amyloid polyneuropathy2011
Author(s)
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Organizer
2011 Biennial Meeting of the Peripheral Nerve Society
Place of Presentation
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[Presentation] Effect of Liver Transplantation on Peripheral Neuropathy of Familial Amyloid Polyneuropathy V30M2011
Author(s)
Yamashita T, Oshima T, Okumura K, Nagatoshi A, Misumi M, Okamoto S, Ueda A, Ueda M, Obayashi K, Nakamura M, Jono H, Shono M, Asonuma K, Ando Y, Inomata Y
Organizer
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Author(s)
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Organizer
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Place of Presentation
熊本市医師会館, 熊本県熊本市
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Author(s)
安東由喜雄, 山下太郎, 大嶋俊範, 奥村幸祐, 永利聡仁, 三隅洋平, 田崎雅, 神力 悟, 植田光晴, 大林光念, 庄野 信, 城野博史
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