CIPSM - Telethonin Deficiency Is Associated With Maladaptation to Biomechanical Stress in the Mammalian Heart

Telethonin Deficiency Is Associated With Maladaptation to Biomechanical Stress in the Mammalian Heart

28-Jul-2011

Ralph Knöll, Wolfgang A. Linke, Peijian Zou, Snježana Miočiċ, Sawa Kosti Byambajav Buyandelger, Ching-Hsin Ku, Stefan Neef, Monika Bug, Katrin Schäfer, Gudrun Knöll, Leanne E. Felkin, Johannes Wessels, Karl Toischer, Franz Hagn, Horst Kessler, Michael Didié, Thomas Quentin, Lars S. Maier, Nils Teucher, Bernhard Unsöld, Albrecht Schmidt, Emma J. Birks, Sylvia Gunkel, Patrick Lang, Henk Granzier, Wolfram-Hubertus Zimmermann, Loren J. Field, Georgine Faulkner, Matthias Dobbelstein, Paul J.R. Barton, Michael Sattler, Matthias Wilmanns, Kenneth R. Chien

Circulation Research, 2011, doi: 10.1161/CIRCRESAHA.111.245787, 758-769 published on 28.07.2011

Circulation Research, online article

Telethonin (also known as titin-cap or t-cap) is a 19-kDa Z-disk protein with a unique beta-sheet structure, hypothesized to assemble in a palindromic way with the N-terminal portion of titin and to constitute a signalosome participating in the process of cardiomechanosensing. In addition, a variety of telethonin mutations are associated with the development of several different diseases; however, little is known about the underlying molecular mechanisms and telethonin’s in vivo function.