A Disintegrin and Metalloproteinase with Thrombospondin Motifs-like-5 (adamtsl-5) Is a Novel Extracellular Matrix Protein That Binds to Fibrillin-1 and Promotes Fibrillin-1 Fibril Formation*
نویسندگان
چکیده
From 1 Sekiguchi Biomatrix Signaling Project, Exploratory Research for Advanced Technology, Japan Science and Technology Agency, c/o Aichi Medical University, Nagakute, Aichi 480-1195, Japan and 2 Institute for Protein Research, Osaka University, Suita, Osaka 565-0871, Japan and 3 Shriners Hospital for Children and 4 Department of Biochemistry and Molecular Biology, Oregon Health & Science University, Portland, OR 97239, USA Running title: ADAMTSL-5 promotes fibrillin-1 microfibril assembly Address correspondence to: Kiyotoshi Sekiguchi, Institute for Protein Research, Osaka University, 3-2 Yamadaoka, Suita, Osaka 565-0871, Japan. Fax: +81-6-6879-8619; E-mail: [email protected]
منابع مشابه
The Journal of Biological Chemistry ADAMTSL6β rescues disorder in Marfan syndrome Saito et al, Page1 ADAMTSL6β rescues fibrillin-1 microfibril disorder in a Marfan syndrome mouse model through the promotion of fibrillin-1 assembly
Marfan’s 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. A disintegrinlike metalloprotease domain with thrombospondin type I motifs like (ADAMTSL) 6β is a microfibril-associated extracellular matrix protein expressed in ...
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