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Forelimb Contractures and Abnormal Tendon Collagen Fibrillogenesis in Fibulin-4 Null Mice

Fibulin-4 is an extracellular matrix glycoprotein essential for elastic fiber formation. Mice deficient in fibulin-4 die perinatally due to severe pulmonary and vascular defects associated with lacking intact elastic fibers. Patients with fibulin-4 mutations demonstrate similar defects and a signifi...

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Detalhes bibliográficos
Publicado no:Cell Tissue Res
Main Authors: Markova, Dessislava Z., Pan, Te-Cheng, Zhang, Rui-Zhu, Zhang, Guiyun, Sasaki, Takako, Arita, Machiko, Birk, David E., Chu, Mon-Li
Formato: Artigo
Idioma:Inglês
Publicado em: 2015
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4876057/
https://ncbi.nlm.nih.gov/pubmed/26711913
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s00441-015-2346-x
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