Adamtsl2 deletion results in bronchial fibrillin microfibril accumulation and bronchial epithelial dysplasia – a novel mouse model providing insights into geleophysic dysplasia

Mutations in the secreted glycoprotein ADAMTSL2 cause recessive geleophysic dysplasia (GD) in humans and Musladin–Lueke syndrome (MLS) in dogs. GD is a severe, often lethal, condition presenting with short stature, brachydactyly, stiff skin, joint contractures, tracheal-bronchial stenosis and cardia...

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Detalles Bibliográficos
Autores principales: Hubmacher, Dirk, Wang, Lauren W., Mecham, Robert P., Reinhardt, Dieter P., Apte, Suneel S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists 2015
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4415891/
https://www.ncbi.nlm.nih.gov/pubmed/25762570
http://dx.doi.org/10.1242/dmm.017046

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4415891/
https://www.ncbi.nlm.nih.gov/pubmed/25762570
http://dx.doi.org/10.1242/dmm.017046

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