Academy of Finland
Funding decision
Applicant / Contact person Taimen, Pekka
Organisation University of Turku
Project title LMNA-related dilated cardiomyopathy: modelling and molecular targeting of cellular stress in vitro and in vivo
Decision No. 309443
Decision date 28.04.2017
Funding period 01.09.2017 - 31.08.2021
Funding 448 849
WebFOCUS Report
Project description
Dilated cardiomyopathy (DCM) is a progressive cardiac disease characterized by reduced pump function and dilatation of heart. In a third of the cases the disease is genetic. The second most common gene affected is LMNA encoding nuclear lamins A and C. Lamins are filament proteins that determine size and shape of the cell nucleus and contribute many essential cell functions such as regulation of gene expression. However, it is currently unknown how mutations in lamins eventually lead to DCM. In this project we model cellular and molecular alterations caused by mutant lamin in human stem cell derived cardiac cells and in a experimental fruit fly model. The ultimate goal is to identify potential drug targets to develop new therapies for this progressive and devastating disease.