Articles with "fibrillin" as a keyword



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Unusual life cycle and impact on microfibril assembly of ADAMTS17, a secreted metalloprotease mutated in genetic eye disease

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Published in 2017 at "Scientific Reports"

DOI: 10.1038/srep41871

Abstract: Secreted metalloproteases have diverse roles in the formation, remodeling, and the destruction of extracellular matrix. Recessive mutations in the secreted metalloprotease ADAMTS17 cause ectopia lentis and short stature in humans with Weill-Marchesani-like syndrome and primary… read more here.

Keywords: fibrillin; unusual life; life cycle; secreted metalloprotease ... See more keywords
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Fibrillin-1 regulates endothelial sprouting during angiogenesis.

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Published in 2023 at "Proceedings of the National Academy of Sciences of the United States of America"

DOI: 10.1073/pnas.2221742120

Abstract: Fibrillin-1 is an extracellular matrix protein that assembles into microfibrils which provide critical functions in large blood vessels and other tissues. Mutations in the fibrillin-1 gene are associated with cardiovascular, ocular, and skeletal abnormalities in… read more here.

Keywords: cell; fibrillin; matrix; endothelial sprouting ... See more keywords