{"created":"2021-03-01T06:09:32.883377+00:00","id":5681,"links":{},"metadata":{"_buckets":{"deposit":"58cd7962-ba6f-45d4-a3b9-a28a78c7f692"},"_deposit":{"id":"5681","owners":[],"pid":{"revision_id":0,"type":"depid","value":"5681"},"status":"published"},"_oai":{"id":"oai:niigata-u.repo.nii.ac.jp:00005681","sets":["453:455","471:561:562"]},"item_6_alternative_title_1":{"attribute_name":"その他のタイトル","attribute_value_mlt":[{"subitem_alternative_title":"Heterogeneity of cortical TDP-43 pathology in amyotrophic lateral sclerosis : Clinicopathological analysis"}]},"item_6_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2016-03-23","bibliographicIssueDateType":"Issued"},"bibliographic_titles":[{}]}]},"item_6_date_granted_51":{"attribute_name":"学位授与年月日","attribute_value_mlt":[{"subitem_dategranted":"2016-03-23"}]},"item_6_degree_grantor_49":{"attribute_name":"学位授与機関","attribute_value_mlt":[{"subitem_degreegrantor":[{"subitem_degreegrantor_name":"新潟大学"}]}]},"item_6_degree_name_48":{"attribute_name":"学位名","attribute_value_mlt":[{"subitem_degreename":"博士（医学）"}]},"item_6_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"筋萎縮性側索硬化症 (amyotrophic lateral sclerosis: ALS) は，上位および下位運動ニューロンが侵されることにより，進行性に筋力低下を来す神経変性疾患である．近年，ALSとユビキチン陽性かつタウおよびα-シヌクレイン陰性の細胞内封入体を持つ前頭側頭葉変性症(frontotemporal lobar degeneration with ubiquitin inclusions: FTLD-U) に共通する疾患原因蛋白としてTAR DNA-binding protein of 43kDa (TDP-43) が同定され，両疾患はTDP-43 proteinopathy という新たな疾患群としてとらえられている．FTLD-TDPは大脳皮質におけるTDP-43陽性組織像に基づき4タイプに分類されている．しかし，ALSにおけるそれは明らかでない．本研究では，孤発性ALS連続剖検例96例を対象として，抗リン酸化TDP-43抗体 (phosphorylated TDP-43: pTDP-43) を用い，大脳皮質および皮質下諸核のpTDP-43陽性組織像を検討した．まず，海馬歯状回顆粒細胞におけるpTDP-43陽性神経細胞質内封入体 (neuronal cytoplasmic inclusions: NCIs) の有無に注目し，それを欠くtype1群 (n = 63) とそれを有するtype 2群 (n = 33) に分類した．さらに，type 2群の症例の中には，側頭葉新皮質に無数の顆粒状，点状DNsが見られる症例とそれをほとんど見ない症例が存在したため，側頭葉新皮質におけるDNs の出現の多寡により， type 2群をsparse DNs群 (n= 22) とabundant DNs群 (n= 11) に分類した．これら3 群について，臨床病理学的解析を行った．臨床的には，type 1 群では認知症の合併はきわめてまれであった．また，type2/abundant DNs群は他の2 群に比し生命予後が不良であった．一方，組織学的にはtype2/abundant DNs群では下位運動ニューロンの脱落が軽く，大脳皮質と同様に新線条体と淡蒼球にも無数の顆粒状や点状のpTDP-43陽性構造を高率に認めた．免疫蛍光二重染色による検討では，これらは神経樹状突起棘への蓄積であると考えられた．各群の罹病期間や運動ニューロン脱落の程度を考慮すると，疾患の進行に伴い，type 1群からtype 2群へ，あるいはtype 2/sparse DNs群からtype 2/abundant DNs群への移行は考え難く，むしろ，これら3群はpTDP-43の脳内および細胞内局在が異なる独立した亜群であるとみなされた．","subitem_description_type":"Abstract"}]},"item_6_description_5":{"attribute_name":"内容記述","attribute_value_mlt":[{"subitem_description":"学位の種類: 博士（医学）. 報告番号: 甲第4096号. 学位記番号: 新大院博（医）甲第663号. 学位授与年月日: 平成28年3月23日","subitem_description_type":"Other"}]},"item_6_description_53":{"attribute_name":"学位記番号","attribute_value_mlt":[{"subitem_description":"新大院博（医）甲第663号","subitem_description_type":"Other"}]},"item_6_dissertation_number_52":{"attribute_name":"学位授与番号","attribute_value_mlt":[{"subitem_dissertationnumber":"13101甲第4096号"}]},"item_6_full_name_3":{"attribute_name":"著者別名","attribute_value_mlt":[{"nameIdentifiers":[{"nameIdentifier":"50714","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Takeuchi, Ryoko"}]}]},"item_6_publisher_7":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"新潟大学"}]},"item_6_select_19":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_select_item":"ETD"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"竹内, 亮子"}],"nameIdentifiers":[{"nameIdentifier":"50713","nameIdentifierScheme":"WEKO"}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2019-08-05"}],"displaytype":"detail","filename":"h27nmk663.pdf","filesize":[{"value":"2.1 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臨床病理学的解析．"},{"subitem_title":"筋萎縮性側索硬化症におけるTDP-43陽性組織像の多様性 : 臨床病理学的解析．","subitem_title_language":"en"}]},"item_type_id":"6","owner":"1","path":["455","562"],"pubdate":{"attribute_name":"公開日","attribute_value":"2016-05-19"},"publish_date":"2016-05-19","publish_status":"0","recid":"5681","relation_version_is_last":true,"title":["筋萎縮性側索硬化症におけるTDP-43陽性組織像の多様性 : 臨床病理学的解析．"],"weko_creator_id":"1","weko_shared_id":2},"updated":"2022-12-15T03:38:50.095391+00:00"}