Lineage-specific events underlie aortic root aneurysm pathogenesis in Loeys-Dietz syndrome
Lineage-specific events underlie aortic root aneurysm pathogenesis in Loeys-Dietz syndrome
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Author / Creator
MacFarlane, Elena Gallo , Parker, Sarah J , Shin, Joseph Y , Kang, Benjamin E , Ziegler, Shira G , Creamer, Tyler J , Bagirzadeh, Rustam , Bedja, Djahida , Chen, Yichun , Calderon, Juan F , Weissler, Katherine , Frischmeyer-Guerrerio, Pamela A , Lindsay, Mark E , Habashi, Jennifer P and Dietz, Harry C
Publisher
United States: American Society for Clinical Investigation
Journal title
Language
English
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Publisher
United States: American Society for Clinical Investigation
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Scope and Contents
Contents
The aortic root is the predominant site for development of aneurysm caused by heterozygous loss-of-function mutations in positive effectors of the transforming growth factor-β (TGF-β) pathway. Using a mouse model of Loeys-Dietz syndrome (LDS) that carries a heterozygous kinase-inactivating mutation in TGF-β receptor I, we found that the effects of...
Alternative Titles
Full title
Lineage-specific events underlie aortic root aneurysm pathogenesis in Loeys-Dietz syndrome
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Record Identifier
TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_6355234
Permalink
https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_6355234
Other Identifiers
ISSN
0021-9738
E-ISSN
1558-8238
DOI
10.1172/JCI123547
