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

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 thrombosp...

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Enregistré dans:
Détails bibliographiques
Auteurs principaux: 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
Format: Artigo
Langue:Inglês
Publié: American Society for Biochemistry and Molecular Biology 2011
Sujets:
Developmental Biology
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC3207443/
https://ncbi.nlm.nih.gov/pubmed/21880733
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M111.243451
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