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ABSTRACT Geleophysic dysplasia (GD) is a rare genetic condition caused by pathogenic variants in either the ADAMTSL2 or FBN1 gene. We present a patient that combined clinical features consistent with GD, including short stature, delayed bone age, progressive congenital heart disease, and a happy, round face.
Ray Thomas Katbe +2 more
wiley +1 more source
Local complement production by pulmonary artery adventitial fibroblasts, activated intracellularly by CFD and CFB and extracellularly by GZMK+ CD8 T cells, and its secretion in soluble form and within EVs promotes macrophage and T cell chemotaxis and activation.
Hui Zhang +9 more
wiley +1 more source
The Importance of Liver‐Lung Communication in Pulmonary Vascular Diseases
Schematic depicting the key drivers of liver‐lung communication in pulmonary vascular diseases, including hepatic dysfunction, portosystemic shunting, and gut microbial dysbiosis, which together drive pulmonary vascular remodeling. ABSTRACT In normal health, the liver and lungs enjoy a close anatomic, physiologic, and functional relationship.
Navneet Singh +8 more
wiley +1 more source
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Journal of Cellular Physiology, 2014
Our previous work showed that the expression of bone morphogenetic protein‐4 (BMP4) was up‐regulated in pathological cardiac hypertrophy models and BMP4 induced cardiomyocyte hypertrophy and apoptosis. Bone morphogenetic protein‐2 (BMP2) and BMP4 share greater than 80% amino acid homology and there exists an interaction between BMP2 and BMP4, so the ...
Jing Lu +8 more
semanticscholar +3 more sources
Our previous work showed that the expression of bone morphogenetic protein‐4 (BMP4) was up‐regulated in pathological cardiac hypertrophy models and BMP4 induced cardiomyocyte hypertrophy and apoptosis. Bone morphogenetic protein‐2 (BMP2) and BMP4 share greater than 80% amino acid homology and there exists an interaction between BMP2 and BMP4, so the ...
Jing Lu +8 more
semanticscholar +3 more sources
Bone morphogenetic protein 4 inhibits liposaccharide‐induced inflammation in the airway
European Journal of Immunology, 2014Bone morphogenetic protein 4 (BMP4) is a multifunctional growth factor that belongs to the TGF‐β superfamily. The role of BMP4 in lung diseases is not fully understood. Here, we demonstrate that BMP4 was upregulated in lungs undergoing lipopolysaccharide (LPS)‐induced inflammation, and in airway epithelial cells treated with LPS or TNF‐α.
Zhengtu Li +13 more
semanticscholar +3 more sources
Bone morphogenetic protein 4 alleviates pulmonary fibrosis by regulating macrophages
International Immunopharmacology, 2023Fibrosis is a pathological change mainly characterized by an increase of fibrous connective tissue and decrease of parenchymal cells. Its continuous progress may lead to the destruction of organ structure and function decline. An excess of alternatively activated M2 macrophages have been considered crucial candidates in the progression of fibrosis ...
Yiyang Jiang +5 more
openaire +2 more sources
The International Journal of Biochemistry & Cell Biology, 1996
Bone morphogenic protein-4 (BMP-4) is one of nine structurally related BMPs belonging to the transforming growth factor-beta (TGF-beta) superfamily of secreted proteins. Mature BMP-4 is a dimer that binds to a multimeric transmembrane receptor with serine/threonine kinase activity.
L M, Leong, P M, Brickell
openaire +2 more sources
Bone morphogenic protein-4 (BMP-4) is one of nine structurally related BMPs belonging to the transforming growth factor-beta (TGF-beta) superfamily of secreted proteins. Mature BMP-4 is a dimer that binds to a multimeric transmembrane receptor with serine/threonine kinase activity.
L M, Leong, P M, Brickell
openaire +2 more sources
The Role of Bone Morphogenetic Protein 4 in Lung Diseases
Current Molecular Medicine, 2023: Bone morphogenetic protein 4 (BMP4) is a multifunctional secretory protein that belongs to the transforming growth factor β superfamily. BMPs transduce their signaling to the cytoplasm by binding to membrane receptors of the serine/threonine kinase family, including BMP type I and type II receptors. BMP4 participates in various biological processes,
Xiaoxiao, Dong, Yimin, Mao, Pengfei, Gao
openaire +2 more sources

