Cardiopulmonary dysfunction in the Osteogenesis imperfecta mouse model Aga2 and human patients are caused by bone-independent mechanisms

Osteogenesis imperfecta (OI) is an inherited connective tissue disorder with skeletal dysplasia of varying severity, predominantly caused by mutations in the collagen I genes (COL1A1/COL1A2). Extraskeletal findings such as cardiac and pulmonary complications are generally considered to be significan...

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Autori principali: Thiele, Frank, Cohrs, Christian M., Flor, Armando, Lisse, Thomas S., Przemeck, Gerhard K. H., Horsch, Marion, Schrewe, Anja, Gailus-Durner, Valerie, Ivandic, Boris, Katus, Hugo A., Wurst, Wolfgang, Reisenberg, Catherine, Chaney, Hollis, Fuchs, Helmut, Hans, Wolfgang, Beckers, Johannes, Marini, Joan C., Hrabé de Angelis, Martin
Natura: Artigo
Lingua:Inglês
Pubblicazione: Oxford University Press 2012
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Articles
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC3406754/
https://ncbi.nlm.nih.gov/pubmed/22589248
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/dds183
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