نتایج جستجو برای: titin

تعداد نتایج: 1511  

Journal: :Science 2015
John T Hinson Anant Chopra Navid Nafissi William J Polacheck Craig C Benson Sandra Swist Joshua Gorham Luhan Yang Sebastian Schafer Calvin C Sheng Alireza Haghighi Jason Homsy Norbert Hubner George Church Stuart A Cook Wolfgang A Linke Christopher S Chen J G Seidman Christine E Seidman

Human mutations that truncate the massive sarcomere protein titin [TTN-truncating variants (TTNtvs)] are the most common genetic cause for dilated cardiomyopathy (DCM), a major cause of heart failure and premature death. Here we show that cardiac microtissues engineered from human induced pluripotent stem (iPS) cells are a powerful system for evaluating the pathogenicity of titin gene variants....

Journal: :The Journal of Cell Biology 2002
Nicole LeBrasseur

In This Issue In This Issue Clash of the titin he sarcomere is more than a complicated piece of structural machinery. According to new results by McElhinny et al. (page 125), a building block of striated muscle cells called MURF-1 may also indirectly regulate gene expression. MURF-1 localizes to sarcomeres thanks to its interaction with titin, a major structural component of the muscle sarcomer...

Journal: :Brain : a journal of neurology 2014
Carola Hedberg Atle Melberg Kathe Dahlbom Anders Oldfors

Sir, Hereditary myopathy with early respiratory failure (HMERF) is a neuromuscular disease associated with aggregation of various proteins in muscle fibres and muscle degeneration (Fig. 1) and was described in detail in several families by Edström et al. (1990). Linkage analyses indicated that the disease locus was in the distal part of the long arm of chromosome 2 (Nicolao et al., 1999). Titin...

Journal: :Circulation 2011
Kalkidan Bishu Nazha Hamdani Selma F Mohammed Martina Kruger Tomohito Ohtani Ozgur Ogut Frank V Brozovich John C Burnett Wolfgang A Linke Margaret M Redfield

BACKGROUND In vitro studies suggest that phosphorylation of titin reduces myocyte/myofiber stiffness. Titin can be phosphorylated by cGMP-activated protein kinase. Intracellular cGMP production is stimulated by B-type natriuretic peptide (BNP) and degraded by phosphodiesterases, including phosphodiesterase-5A. We hypothesized that a phosphodiesterase-5A inhibitor (sildenafil) alone or in combin...

Journal: :Circulation Research 2009

2014
Anna E. Müller Matthias Kreiner Sebastian Kötter Philipp Lassak Wilhelm Bloch Frank Suhr Martina Krüger

Titin-based myofilament stiffness is largely modulated by phosphorylation of its elastic I-band regions N2-Bus (decreases passive stiffness, PT) and PEVK (increases PT). Here, we tested the hypothesis that acute exercise changes titin phosphorylation and modifies myofilament stiffness. Adult rats were exercised on a treadmill for 15 min, untrained animals served as controls. Titin phosphorylati...

Journal: :The Journal of Cell Biology 1989
R Nave D O Fürst K Weber

TII, the extractable form of titin, was purified from myofibrils and separated by high resolution gel permeation chromatography into two fractions (TIIA and TIIB). Novel specimen orientation methods used before metal shadowing and EM result in striking pictures of the two forms. Molecules layered on mica become uniformly oriented when subjected to centrifugation. TIIB comprises a very homogeneo...

Journal: :The Journal of General Physiology 2003
Michiel Helmes Chee Chew Lim Ronglih Liao Ajit Bharti Lei Cui Douglas B. Sawyer

Titin, a giant protein spanning half the sarcomere, is responsible for passive and restoring forces in cardiac myofilaments during sarcomere elongation and compression, respectively. In addition, titin has been implicated in the length-dependent activation that occurs in the stretched sarcomere, during the transition from diastole to systole. The purpose of this study was to investigate the rol...

2012
Mike DuVall Azim Jinha Tim Leonard Walter Herzog

INTRODUCTION Within skeletal and cardiac muscle exists a molecular spring responsible for maintaining muscle structure, preventing stretch related damage, and contributing to the development of muscle force. This spring, called titin, has almost exclusively been associated with passive (elastic) force, that develops when muscles are stretched. However, more recent work has suggested there may b...

Journal: :COVID 2022

During the SARS-CoV-2 (COVID-19) pandemic, immunogenicity of virus for various autoimmune complications has been observed. To date, a few reports have published that raise possibility new onset myasthenia gravis (MG) associated with COVID-19 infection. We report case 65-year-old male who developed his initial myasthenic presentation mild dysarthria 14 days after infection symptomatic onset. His...

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