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Pericytes as targets in hereditary hemorrhagic telangiectasia
- Source :
- Frontiers in Genetics, Vol 6 (2015), Frontiers in Genetics
- Publication Year :
- 2015
- Publisher :
- Frontiers Media S.A., 2015.
-
Abstract
- Defective paracrine Transforming Growth Factor-β (TGF-β) signaling between endothelial cells and the neighboring mural cells have been thought to lead to the development of vascular lesions that are characteristic of Hereditary Hemorrhagic Telangiectasia (HHT). This review highlights recent progress in our understanding of TGF-β signaling in mural cell recruitment and vessel stabilization and how perturbed TGF-β signaling might contribute to defective endothelial-mural cell interaction affecting vessel functionalities. Our recent findings have provided exciting insights into the role of thalidomide, a drug that reduces both the frequency and the duration of epistaxis in individuals with HHT by targeting mural cells. These advances provide opportunities for the development of new therapies for vascular malformations.
- Subjects :
- transforming growth factor-β signaling
lcsh:QH426-470
Angiogenesis
Cell
Review Article
Bioinformatics
Pediatrics
Mural cell
Paracrine signalling
angiogenesis
pericyte
thalidomide
Genetics
Medicine
Telangiectasia
rare vascular disease
Genetics (clinical)
business.industry
Thalidomide
lcsh:Genetics
medicine.anatomical_structure
Cancer research
cardiovascular system
Molecular Medicine
Pericyte
medicine.symptom
business
medicine.drug
Transforming growth factor
Subjects
Details
- Language :
- English
- ISSN :
- 16648021
- Volume :
- 6
- Database :
- OpenAIRE
- Journal :
- Frontiers in Genetics
- Accession number :
- edsair.doi.dedup.....eac98a4f4f31d0c452e9b71793b763d0
- Full Text :
- https://doi.org/10.3389/fgene.2015.00037