{"created":"2023-06-19T10:44:00.684785+00:00","id":4378,"links":{},"metadata":{"_buckets":{"deposit":"fc687fee-60c8-4f02-b3d1-64a3008a9ccd"},"_deposit":{"created_by":11,"id":"4378","owners":[11],"pid":{"revision_id":0,"type":"depid","value":"4378"},"status":"published"},"_oai":{"id":"oai:shiga-med.repo.nii.ac.jp:00004378","sets":["30:198:199"]},"author_link":["7331","1956"],"item_4_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographic_titles":[{},{"bibliographic_title":"International Journal of Molecular Sciences","bibliographic_titleLang":"en"}]}]},"item_4_creator_3":{"attribute_name":"著者別名","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"漆谷, 真"}],"nameIdentifiers":[{},{},{}]}]},"item_4_description_14":{"attribute_name":"フォーマット","attribute_value_mlt":[{"subitem_description":"application/pdf","subitem_description_type":"Other"}]},"item_4_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"TAR DNA binding protein 43 (TDP-43) is a DNA/RNA binding protein involved in pivotal cellular functions, especially in RNA metabolism. Hyperphosphorylated and ubiquitinated TDP-43-positive neuronal cytoplasmic inclusions are identified in the brain and spinal cord in most cases of amyotrophic lateral sclerosis (ALS) and a substantial proportion of frontotemporal lobar degeneration (FTLD) cases. TDP-43 dysfunctions and cytoplasmic aggregation seem to be the central pathogenicity in ALS and FTLD. Therefore, unraveling both the physiological and pathological mechanisms of TDP-43 may enable the exploration of novel therapeutic strategies. This review highlights the current understanding of TDP-43 biology and pathology, describing the cellular processes involved in the pathogeneses of ALS and FTLD, such as post-translational modifications, RNA metabolism, liquid-liquid phase separation, proteolysis, and the potential prion-like propagation propensity of the TDP-43 inclusions. ","subitem_description_type":"Abstract"}]},"item_4_description_42":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"subitem_description":"Journal Article","subitem_description_type":"Other"}]},"item_4_publisher_32":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"MDPI"}]},"item_4_relation_10":{"attribute_name":"PubMed番号","attribute_value_mlt":[{"subitem_relation_type":"isIdenticalTo","subitem_relation_type_id":{"subitem_relation_type_id_text":"36293362","subitem_relation_type_select":"PMID"}}]},"item_4_relation_11":{"attribute_name":"DOI","attribute_value_mlt":[{"subitem_relation_name":[{"subitem_relation_name_text":"10.3390/ijms232012508"}],"subitem_relation_type":"isIdenticalTo","subitem_relation_type_id":{"subitem_relation_type_id_text":"https://doi.org/10.3390/ijms232012508","subitem_relation_type_select":"DOI"}}]},"item_4_relation_13":{"attribute_name":"PMCID","attribute_value_mlt":[{"subitem_relation_name":[{"subitem_relation_name_text":"PMC9604209"}],"subitem_relation_type_id":{"subitem_relation_type_id_text":"http://www.ncbi.nlm.nih.gov/pmc/articles/pmc9604209/","subitem_relation_type_select":"URI"}}]},"item_4_rights_12":{"attribute_name":"権利","attribute_value_mlt":[{"subitem_rights":"© 2022 by the authors. Licensee MDPI, Basel, Switzerland."}]},"item_4_source_id_7":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"1661-6596","subitem_source_identifier_type":"ISSN"}]},"item_4_version_type_15":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_version_resource":"http://purl.org/coar/version/c_970fb48d4fbd8a85","subitem_version_type":"VoR"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"TAMAKI, Yoshitaka"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"URUSHITANI, Makoto"}],"nameIdentifiers":[{},{},{}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2022-11-21"}],"displaytype":"detail","filename":"ijms232012508.pdf","filesize":[{"value":"5.9 MB"}],"format":"application/pdf","license_note":"This is an open access article distributed under the Creative Commons \nAttribution License which permits unrestricted use, distribution, and \nreproduction in any medium, provided the original work is properly cited. \n","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"ijms232012508","url":"https://shiga-med.repo.nii.ac.jp/record/4378/files/ijms232012508.pdf"},"version_id":"e6180af8-7d37-4981-bd42-6dacc0e95b7d"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"TDP-43","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"RNA metabolism","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"aggregation","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"phase separation","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"neurodegenerative diseases","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"ALS","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"FTLD","subitem_subject_language":"en","subitem_subject_scheme":"Other"}]},"item_language":{"attribute_name":"言語","attribute_value_mlt":[{"subitem_language":"eng"}]},"item_resource_type":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"resourcetype":"journal article","resourceuri":"http://purl.org/coar/resource_type/c_6501"}]},"item_title":"Molecular Dissection of TDP-43 as a Leading Cause of ALS/FTLD","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"Molecular Dissection of TDP-43 as a Leading Cause of ALS/FTLD"}]},"item_type_id":"4","owner":"11","path":["199"],"pubdate":{"attribute_name":"公開日","attribute_value":"2022-11-21"},"publish_date":"2022-11-21","publish_status":"0","recid":"4378","relation_version_is_last":true,"title":["Molecular Dissection of TDP-43 as a Leading Cause of ALS/FTLD"],"weko_creator_id":"11","weko_shared_id":-1},"updated":"2023-06-19T11:09:43.971536+00:00"}