HYPER-FIBRO

Cardiac fibrosis in hypertrophic cardiomyopathy: elucidating the role of cardiac fibroblasts

  • Project duration: December 2020 - December 2023
  • Project status:
    Ongoing
  • Funding:
    Provincial P.-L.P. 14. Mobility (Province BZ funding /Project)

Hypertrophic cardiomyopathy (HCM) is one of the most common genetic heart diseases and the leading cause of sudden cardiac death in young people. Cardiac fibrosis, the excess deposition of extracellular matrix protein (ECM), is an important histologic feature of HCM that may interfere with the electrical conductance of the heart and accelerate the progression of heart failure. In the centre of this fibrotic process are the cardiac fibroblast (cFB) that - in response to pathological stimuli - proliferate, migrate and differentiate into myofibroblasts (MyoFB), the predominant ECM-synthesizing cells. The molecular mechanisms involved in cardiac fibrosis in HCM are incompletely understood and to date, there is no effective treatment to attenuate the fibrotic process. TGF-β-signalling and dysregulated intracellular Ca2+ handling both play a key role for the differentiation of cFB into MyoFB and may therefore represent a relevant therapeutic target for cardiac fibrosis in HCM. The overall aim of this project is to elucidate the molecular and cellular mechanisms that lead to the development of cardiac fibrosis in HCM in primary human cFB. The specific objectives are to study cFB dysfunction with respect to their proliferation, migration and differentiation; to establish the molecular basis of pathways involved in TGF-β-induced fibrosis; and to determine the role of altered intracellular Ca2+ signalling for fibrogenesis in HCM. The project will be completed over a total duration of 24 months.

Partner
Università degli Studi di Firenze
Project Team
1 - 2

Projects

1 - 4
Project

EPIC

Epigenetics of Immunity in Cancer

Duration: September 2019 - August 2021Funding:
Italy-Austria 2014-2020 (EUTC / EU funding / Project)

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Eurac Research is a private research center based in Bolzano (South Tyrol) with researchers from a wide variety of scientific fields who come from all over the globe. Together, through scientific knowledge and research, they share the goal of shaping the future.

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