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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Main Authors: Dirk Hubmacher (Author), Lauren W. Wang (Author), Robert P. Mecham (Author), Dieter P. Reinhardt (Author), Suneel S. Apte (Author)
Format: Book
Published: The Company of Biologists, 2015-05-01T00:00:00Z.
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3rd Floor Main Library

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Call Number: A1234.567
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