TBX18 | Transcriptional regulation by TBX18 in vascular development and disease

Summary
Mural cells (pericytes and vascular smooth muscle) enclose blood vessels and are critical for vascular homeostasis.
Absence or malfunction of pericytes or vascular smooth muscle results in aneurysm formation in small or large blood
vessels, respectively. Our previous work showed Tbx18 is selectively expressed in mural cells of multiple adult organs.
Preliminary data indicates that, in mice, ablation of Tbx18 in mural cells results in aortic tortuosity and lethality due to
rupture. These observations led to the hypothesis that transcriptional regulation by TBX18 in mural cells is critical for the
development of functional vascular networks. To fully understand the roles of TBX18 in mural cells and eventually place it
as a gene involved in human vascular disease, we propose to: 1) fully characterize the vascular phenotypes of Pdgfrb-
Cre;Tbx18 mutants; 2) identify genes directly regulated by Tbx18 in mural cells; and 3) test a putative involvement of TBX18
in human aneurysmal diseases.
Unfold all
/
Fold all
More information & hyperlinks
Web resources: https://cordis.europa.eu/project/id/748035
Start date: 02-01-2018
End date: 01-01-2020
Total budget - Public funding: 168 277,20 Euro - 168 277,00 Euro
Cordis data

Original description

Mural cells (pericytes and vascular smooth muscle) enclose blood vessels and are critical for vascular homeostasis.
Absence or malfunction of pericytes or vascular smooth muscle results in aneurysm formation in small or large blood
vessels, respectively. Our previous work showed Tbx18 is selectively expressed in mural cells of multiple adult organs.
Preliminary data indicates that, in mice, ablation of Tbx18 in mural cells results in aortic tortuosity and lethality due to
rupture. These observations led to the hypothesis that transcriptional regulation by TBX18 in mural cells is critical for the
development of functional vascular networks. To fully understand the roles of TBX18 in mural cells and eventually place it
as a gene involved in human vascular disease, we propose to: 1) fully characterize the vascular phenotypes of Pdgfrb-
Cre;Tbx18 mutants; 2) identify genes directly regulated by Tbx18 in mural cells; and 3) test a putative involvement of TBX18
in human aneurysmal diseases.

Status

CLOSED

Call topic

MSCA-IF-2016

Update Date

28-04-2024
Images
No images available.
Geographical location(s)
Structured mapping
Unfold all
/
Fold all
Horizon 2020
H2020-EU.1. EXCELLENT SCIENCE
H2020-EU.1.3. EXCELLENT SCIENCE - Marie Skłodowska-Curie Actions (MSCA)
H2020-EU.1.3.2. Nurturing excellence by means of cross-border and cross-sector mobility
H2020-MSCA-IF-2016
MSCA-IF-2016