Structural elements of muscle
P7
Subproject Speakers
Berlin: Michael Gotthardt, MD, Prof.
Paris: Gisèle Bonne, PhD
The giant muscle protein titin functions as elastic scaffold to aid the orderly assembly of the sarcomere and determines the biomechanical properties of muscle. Both features depend on titin's protein domain composition, which is regulated by alternative splicing. Recently, additional functions for titin have been proposed based on its interaction with metabolic enzymes and proteins involved in signal transduction, as well as the titin kinase (TK) domain. The TK domain resides at titin's M-band and shows homology to the serine/threonine kinase domain of smooth muscle myosin-light chain kinase (MLCK) and the invertebrate muscle proteins twitching and stretching-MLCK. In our preliminary studies we used mouse genetics to address titin's role in cardiac signal transduction in vivo and uncovered crosstalk between adrenergic and titin signaling at the level of Ca2+ handling. Here, we will build on the data obtained in our conditional knockout of titin's M-band region that mimics the phenotype of human patients described by Dr. Ferreiro.
Completed theses
|
Students |
MyoGrad PhD student from |
Thesis project |
Defence and degree |
|---|---|---|---|
| Maria Chatzifrangkeskou | 2013-2016 |
Roles of ERK1/2 signaling in LMNA-cardiomyopathy |
Defence on Nov 8, 2016 in Paris Joint doctoral degree from Université Pierre et Marie Curie (UPMC), Paris 6 and Freie Universität Berlin |
| Laurianne Davignon | 2011-2014 | Identification and characterization of new genes associated to multiminicore disease | Defence on Jan 20, 2015 in Paris Joint doctoral degree from Université Pierre et Marie Curie (UPMC), Paris 6 and Freie Universität Berlin |
| Nada Essawy | 2015-2018 | Characterization of emerin LEM-domain missense mutations present in patients with exclusive atrial cardiac defects |
Defence on Dec 12, 2018 in Berlin |
| Martina Fischer | 2013-2017 | Mechanosensing defects and YAP-signaling in LMNA-related congenital muscular dystrophy | Defence on June 28, 2017 in Paris Joint doctoral degree from Université Pierre et Marie Curie (UPMC), Paris 6 and Freie Universität Berlin |
| Coline Macquart | 2014-2017 | Role of the gap junctional protein connexin-43 in the cardiac disease caused by mutations in the gene encoding A-type lamins | Defence on Dec 14, 2017 in Paris Joint doctoral degree from Université Pierre et Marie Curie (UPMC), Paris 6 and Freie Universität Berlin |
| Christopher Polack | 2010-2015 | Function of titin in striated muscles in health and disease | Defence on Aug 31, 2015 in Berlin Doctoral degree from Freie Universität Berlin |
| Christine Schwartz | 2012-2016 | Muscle LIM Protein and Nesprin-1 in mechanotransduction | Defence on Sep 29, 2016 in Berlin Joint doctoral degree from Université Pierre et Marie Curie (UPMC), Paris 6 and Freie Universität Berlin |
| Esma Ziat | 2011-2015 | Emery-Dreifuss muscular dystrophy-associated FHL1 gene mutations: study of molecular and functional consequences in skeletal muscle | Defence on Oct 14, 2015 in Paris Doctoral degree from Université Pierre et Marie Curie (UPMC), Paris 6 |
