ADAMTSL6β Protein Rescues Fibrillin-1 Microfibril Disorder in a Marfan Syndrome Mouse Model through the Promotion of Fibrillin-1 Assembly

Saito, Masahiro; Kurokawa, Misaki; Oda, Masahito; Oshima, Masamitsu; Tsutsui, Ko; Kosaka, Kazutaka; Nakao, Kazuhisa; Ogawa, Miho; Manabe, Ri-ichiroh; Suda, Naoto; Ganjargal, Ganburged; Hada, Yasunobu; Noguchi, Toshihide; Teranaka, Toshio; Sekiguchi, Kiyotoshi; Yoneda, Toshiyuki; Tsuji, Takashi

doi:https://doi.org/10.1074/jbc.M111.243451

ADAMTSL6β Protein Rescues Fibrillin-1 Microfibril Disorder in a Marfan Syndrome Mouse Model through the Promotion of Fibrillin-1 Assembly

Saito, Masahiro; Kurokawa, Misaki; Oda, Masahito et al.

Journal of Biological Chemistry, 2011, 286(44), 38602-38613

アクセス数:1,271件（2025-07-07 11:40 集計）

固定URL: https://hdl.handle.net/11094/71419

閲覧可能ファイル

ファイル	フォーマット	利用条件	サイズ	閲覧回数	利用開始日	説明	information
JBC_2011_38602_13	pdf	なし	9.62 MB	284	2019.03.20

論文情報

ファイル出力 EndNote Basic出力 Mendeley出力

タイトル	ADAMTSL6β Protein Rescues Fibrillin-1 Microfibril Disorder in a Marfan Syndrome Mouse Model through the Promotion of Fibrillin-1 Assembly
著者	著者名 Saito, Masahiro
	著者名 Kurokawa, Misaki
	著者名 Oda, Masahito
	NRID 1000000548307 1000000548307 著者名 Oshima, Masamitsu
	著者名 Tsutsui, Ko
	著者名 Kosaka, Kazutaka
	著者名 Nakao, Kazuhisa
	著者名 Ogawa, Miho
	NRID 1000030280837 1000030280837 著者名 Manabe, Ri-ichiroh
	著者名 Suda, Naoto
	著者名 Ganjargal, Ganburged
	著者名 Hada, Yasunobu
	著者名 Noguchi, Toshihide
	NRID 1000060104460 1000060104460 著者名 Teranaka, Toshio
	NRID 1000050187845 1000050187845 researchmap permalink read0054833 read0054833 Scopus Author ID 7201662709 7201662709 ORCID 0000-0002-6433-1410 0000-0002-6433-1410 著者名 Sekiguchi, Kiyotoshi
	NRID 1000080142313 1000080142313 著者名 Yoneda, Toshiyuki
	NRID 1000050339131 1000050339131 著者名 Tsuji, Takashi
内容	This research was originally published in the Journal of Biological Chemistry. Masahiro Saito, Misaki Kurokawa, Masahito Oda, Masamitsu Oshima, Ko Tsutsui, Kazutaka Kosaka, Kazuhisa Nakao, Miho Ogawa, Ri-ichiroh Manabe, Naoto Suda, Ganburged Ganjargal, Yasunobu Hada, Toshihide Noguchi, Toshio Teranaka, Kiyotoshi Sekiguchi, Toshiyuki Yoneda and Takashi Tsuji. ADAMTSL6β Protein Rescues Fibrillin-1 Microfibril Disorder in a Marfan Syndrome Mouse Model through the Promotion of Fibrillin-1 Assembly. J. Biol. Chem. 2011; 286: 38602-38613 © the American Society for Biochemistry and Molecular Biology
抄録	Marfan syndrome (MFS) is a systemic disorder of the connective tissues caused by insufficient fibrillin-1 microfibril formation and can cause cardiac complications, emphysema, ocular lens dislocation, and severe periodontal disease. ADAMTSL6β (A disintegrin-like metalloprotease domain with thrombospondin type I motifs-like 6β) is a microfibril-associated extracellular matrix protein expressed in various connective tissues that has been implicated in fibrillin-1 microfibril assembly. We here report that ADAMTSL6β plays an essential role in the development and regeneration of connective tissues. ADAMTSL6β expression rescues microfibril disorder after periodontal ligament injury in an MFS mouse model through the promotion of fibrillin-1 microfibril assembly. In addition, improved fibrillin-1 assembly in MFS mice following the administration of ADAMTSL6β attenuates the overactivation of TGF-β signals associated with the increased release of active TGF-β from disrupted fibrillin-1 microfibrils within periodontal ligaments. Our current data thus demonstrate the essential contribution of ADAMTSL6β to fibrillin-1 microfibril formation. These findings also suggest a new therapeutic strategy for the treatment of MFS through ADAMTSL6β-mediated fibrillin-1 microfibril assembly.
出版者	American Society for Biochemistry and Molecular Biology
収録物名	Journal of Biological Chemistry
巻(号)	286(44)
ページ	38602-38613
刊行年月	2011-11
言語	英語
ハンドルURL	https://hdl.handle.net/11094/71419
eISSN	1083351X
NCID	AA1202441X
関連情報 (isIdenticalTo)	DOI (出版社版) https://doi.org/10.1074/jbc.M111.243451
アクセス権	open access
出版タイプ	Version of Record
カテゴリ	学術雑誌論文 Journal Article

資源タイプ	学術雑誌論文
ローカル資源タイプ	学術雑誌論文
DCMI資源タイプ	text
DCTERMS.bibliographicCitation	Journal of Biological Chemistry. 2011, 286(44), p. 38602-38613
DC.title	ADAMTSL6β Protein Rescues Fibrillin-1 Microfibril Disorder in a Marfan Syndrome Mouse Model through the Promotion of Fibrillin-1 Assembly
DC.creator	Saito, Masahiro
	Kurokawa, Misaki
	Oda, Masahito
	Oshima, Masamitsu
	Tsutsui, Ko
	Kosaka, Kazutaka
	Nakao, Kazuhisa
	Ogawa, Miho
	Manabe, Ri-ichiroh
	Suda, Naoto
	Ganjargal, Ganburged
	Hada, Yasunobu
	Noguchi, Toshihide
	Teranaka, Toshio
	Sekiguchi, Kiyotoshi
	Yoneda, Toshiyuki
	Tsuji, Takashi
DC.publisher	American Society for Biochemistry and Molecular Biology
DC.language' scheme='DCTERMS.RFC1766	英語
DC.type' scheme='DCTERMS.DCMIType	text
DCTERMS.issued' scheme='DCTERMS.W3CDTF	2011-11
DC.identifier	https://doi.org/10.1074/jbc.M111.243451
DC.description	This research was originally published in the Journal of Biological Chemistry. Masahiro Saito, Misaki Kurokawa, Masahito Oda, Masamitsu Oshima, Ko Tsutsui, Kazutaka Kosaka, Kazuhisa Nakao, Miho Ogawa, Ri-ichiroh Manabe, Naoto Suda, Ganburged Ganjargal, Yasunobu Hada, Toshihide Noguchi, Toshio Teranaka, Kiyotoshi Sekiguchi, Toshiyuki Yoneda and Takashi Tsuji. ADAMTSL6β Protein Rescues Fibrillin-1 Microfibril Disorder in a Marfan Syndrome Mouse Model through the Promotion of Fibrillin-1 Assembly. J. Biol. Chem. 2011; 286: 38602-38613 © the American Society for Biochemistry and Molecular Biology
DCTERMS.abstract	Marfan syndrome (MFS) is a systemic disorder of the connective tissues caused by insufficient fibrillin-1 microfibril formation and can cause cardiac complications, emphysema, ocular lens dislocation, and severe periodontal disease. ADAMTSL6β (A disintegrin-like metalloprotease domain with thrombospondin type I motifs-like 6β) is a microfibril-associated extracellular matrix protein expressed in various connective tissues that has been implicated in fibrillin-1 microfibril assembly. We here report that ADAMTSL6β plays an essential role in the development and regeneration of connective tissues. ADAMTSL6β expression rescues microfibril disorder after periodontal ligament injury in an MFS mouse model through the promotion of fibrillin-1 microfibril assembly. In addition, improved fibrillin-1 assembly in MFS mice following the administration of ADAMTSL6β attenuates the overactivation of TGF-β signals associated with the increased release of active TGF-β from disrupted fibrillin-1 microfibrils within periodontal ligaments. Our current data thus demonstrate the essential contribution of ADAMTSL6β to fibrillin-1 microfibril formation. These findings also suggest a new therapeutic strategy for the treatment of MFS through ADAMTSL6β-mediated fibrillin-1 microfibril assembly.
DC.format	71419
citation_title	ADAMTSL6β Protein Rescues Fibrillin-1 Microfibril Disorder in a Marfan Syndrome Mouse Model through the Promotion of Fibrillin-1 Assembly
citation_author	Saito, Masahiro
	Kurokawa, Misaki
	Oda, Masahito
	Oshima, Masamitsu
	Tsutsui, Ko
	Kosaka, Kazutaka
	Nakao, Kazuhisa
	Ogawa, Miho
	Manabe, Ri-ichiroh
	Suda, Naoto
	Ganjargal, Ganburged
	Hada, Yasunobu
	Noguchi, Toshihide
	Teranaka, Toshio
	Sekiguchi, Kiyotoshi
	Yoneda, Toshiyuki
	Tsuji, Takashi
citation_publisher	American Society for Biochemistry and Molecular Biology
citation_language	英語
citation_date	2011-11
citation_journal_title	Journal of Biological Chemistry
citation_volume	286
citation_issue	44
citation_firstpage	38602
citation_lastpage	38613
citation_doi	https://doi.org/10.1074/jbc.M111.243451
citation_public_url	https://hdl.handle.net/11094/71419

ADAMTSL6β Protein Rescues Fibrillin-1 Microfibril Disorder in a Marfan Syndrome Mouse Model through the Promotion of Fibrillin-1 Assembly

閲覧可能ファイル

論文情報

論文詳細を表示