Ottenheijm, Coen A.C., Witt, Christian C., Stienen, Ger J., Labeit, Siegfried, Beggs, Alan H., Granzier, Henk
Nemaline myopathy (NM) is the most common non-dystrophic congenital myopathy. Clinically the most important feature of NM is muscle weakness; however, the mechanisms underlying this weakness are...
Marie-louise Bang, Ryan E. Mudry, Karoly Trombitás, Adam J. Geach, Rob Yamasaki, Hiroyuki Sorimachi, ...
Abstract. We describe here a novel sarcomeric 145kD protein, myopalladin, which tethers together the COOH-terminal Src homology 3 domains of nebulin and nebulette with the EF hand motifs of...
Fong, Chi, Babu, Gopal J, Periasamy, Muthu, ...
Previous work suggested that altered Ca2+ homeostasis might contribute to dysfunction of nebulin-free muscle, as gene expression analysis revealed that the sarco(endo) plasmic reticulum Ca2+-ATPase...
DeNicola, Gianfelice, Burkart, Christopher, Qiu, Feng, Agianian, Bogos, Labeit, Siegfried, Martin, Stephen, ...
How muscles can be activated by mechanical stretch instead of by calcium efflux sensed through the troponin C (TnC) protein remains unknown. To gain a molecular description of such mechanism, we have...
DeNicola, Gianfelice, Burkart, Christopher, Qiu, Feng, Agianian, Bogos, Labeit, Siegfried, Martin, Stephen, ...
How muscles can be activated by mechanical stretch instead of by calcium efflux sensed through the troponin C (TnC) protein remains unknown. To gain a molecular description of such mechanism, we have...
Adams, Volker, Linke, Axel, Wisloff, Ulrik, Döring, Christian, Erbs, Sandra, Kränkel, Nicolle, ...
Objective: In chronic heart failure (CHF) the myocardial expression of the inflammatory cytokine tumor necrosis factor alpha (TNF-), which is thought to contribute to myocardial remodeling, was found...
Trombit S, Karoly, Greaser, Marion, Labeit, Siegfried, Jin, Jian-Ping, Kellermayer, Mikl S, Helmes, Michiel, ...
Titin (also known as connectin) is a giant protein that spans half of the striated muscle sarcomere. In the I-band titin extends as the sarcomere is stretched, developing what is known as passive...
Fluorescence quenching: A tool for single-molecule protein-folding study
Zhuang, Xiaowei, Ha, Taekjip, Kim, Harold D., Centner, Thomas, Labeit, Siegfried, Chu, Steven
By using titin as a model system, we have demonstrated that fluorescence quenching can be used to study protein folding at the single molecule level. The unfolded titin molecules with multiple dye...
Mutations in the nebulin gene associated with autosomal recessive nemaline myopathy
Pelin, Katarina, Hilpelä, Pirta, Donner, Kati, Sewry, Caroline, Akkari, Patrick A., Wilton, Stephen D., ...
The congenital nemaline myopathies are rare hereditary muscle disorders characterized by the presence in the muscle fibers of nemaline bodies consisting of proteins derived from the Z disc and thin...
Calcium-dependent molecular spring elements in the giant protein titin
Labeit, Dietmar, Watanabe, Kaori, Witt, Christian, Fujita, Hideaki, Wu, Yiming, Lahmers, Sunshine, ...
Titin (also known as connectin) is a giant protein with a wide range of cellular functions, including providing muscle cells with elasticity. Its physiological extension is largely derived from the...
Qiu, Feng, Lakey, Anne, Agianian, Bogos, Hutchings, Amanda, Butcher, Geoffrey W, Labeit, Siegfried, ...
The indirect flight muscles (IFMs) of Lethocerus (giant water bug) and Drosophila (fruitfly) are asynchronous: oscillatory contractions are produced by periodic stretches in the presence of a Ca(2+)...
Fluorescence quenching: A tool for single-molecule protein-folding study
Zhuang, Xiaowei, Ha, Taekjip, Kim, Harold D., Centner, Thomas, Labeit, Siegfried, Chu, Steven
By using titin as a model system, we have demonstrated that fluorescence quenching can be used to study protein folding at the single molecule level. The unfolded titin molecules with multiple dye...
Mutations in the nebulin gene associated with autosomal recessive nemaline myopathy
Pelin, Katarina, Hilpelä, Pirta, Donner, Kati, Sewry, Caroline, Akkari, Patrick A., Wilton, Stephen D., ...
The congenital nemaline myopathies are rare hereditary muscle disorders characterized by the presence in the muscle fibers of nemaline bodies consisting of proteins derived from the Z disc and thin...
Calcium-dependent molecular spring elements in the giant protein titin
Labeit, Dietmar, Watanabe, Kaori, Witt, Christian, Fujita, Hideaki, Wu, Yiming, Lahmers, Sunshine, ...
Titin (also known as connectin) is a giant protein with a wide range of cellular functions, including providing muscle cells with elasticity. Its physiological extension is largely derived from the...
Hackman, Peter, Vihola, Anna, Haravuori, Henna, Marchand, Sylvie, Sarparanta, Jaakko, De Seze, Jerome, ...
Tibial muscular dystrophy (TMD) is an autosomal dominant late-onset distal myopathy linked to chromosome 2q31. The linked region includes the giant TTN gene, which encodes the central sarcomeric...
Qiu, Feng, Lakey, Anne, Agianian, Bogos, Hutchings, Amanda, Butcher, Geoffrey W, Labeit, Siegfried, ...
The indirect flight muscles (IFMs) of Lethocerus (giant water bug) and Drosophila (fruitfly) are asynchronous: oscillatory contractions are produced by periodic stretches in the presence of a Ca(2+)...
Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo
Witt, Christian C, Burkart, Christoph, Labeit, Dietmar, McNabb, Mark, Wu, Yiming, Granzier, Henk, ...
The precise assembly of the highly organized filament systems found in muscle is critically important for its function. It has been hypothesized that nebulin, a giant filamentous protein extending...
Cardiac titin: an adjustable multi-functional spring
Granzier, Henk, Labeit, Siegfried
The giant elastic protein titin contains a molecular spring segment that underlies the majority of myocardial passive stiffness. The mechanical characteristics of this spring may be tuned to match...
Gregorio, Carol C., Trombitás, Karoly, Centner, Thomas, Kolmerer, Bernhard, Stier, Gunter, Kunke, Kathleen, ...
Titin is a giant elastic protein in vertebrate striated muscles with an unprecedented molecular mass of 3–4 megadaltons. Single molecules of titin extend from the Z-line to the M-line. Here, we...
Trombitás, Karoly, Greaser, Marion, Labeit, Siegfried, Jin, Jian-Ping, Kellermayer, Miklós, Helmes, Michiel, ...
Abstract. Titin (also known as connectin) is a giant protein that spans half of the striated muscle sarcomere. In the I-band titin extends as the sarcomere is stretched, developing what is known as...
Linke, Wolfgang A., Rudy, Diane E., Centner, Thomas, Gautel, Mathias, Witt, Christian, Labeit, Siegfried, ...
In cardiac muscle, the giant protein titin exists in different length isoforms expressed in the molecule's I-band region. Both isoforms, termed N2-A and N2-B, comprise stretches of Ig-like modules...
Bang, Marie-Louise, Mudry, Ryan E., McElhinny, Abigail S., Trombitás, Karoly, Geach, Adam J., Yamasaki, Rob, ...
We describe here a novel sarcomeric 145-kD protein, myopalladin, which tethers together the COOH-terminal Src homology 3 domains of nebulin and nebulette with the EF hand motifs of α-actinin in...
McElhinny, Abigail S., Kakinuma, Kazumi, Sorimachi, Hiroyuki, Labeit, Siegfried, Gregorio, Carol C.
The COOH-terminal A168–170 region of the giant sarcomeric protein titin interacts with muscle-specific RING finger-1 (MURF-1). To investigate the functional significance of this interaction, we...
Von Castelmur, Eleonore, Marino, Marco, Svergun, Dmitri I., Kreplak, Laurent, Ucurum-Fotiadis, Zöhre, Konarev, Petr V., ...
Myofibril elasticity, critical to muscle function, is dictated by the intrasarcomeric filament titin, which acts as a molecular spring. To date, the molecular events underlying the mechanics of the...
Cooperative control of striated muscle mass and metabolism by MuRF1 and MuRF2
Witt, Christian C, Witt, Stephanie H, Lerche, Stefanie, Labeit, Dietmar, Back, Walter, Labeit, Siegfried
The muscle-specific RING finger proteins MuRF1 and MuRF2 have been proposed to regulate protein degradation and gene expression in muscle tissues. We have tested the in vivo roles of MuRF1 and MuRF2...
Titin and Diaphragm Dysfunction in Chronic Obstructive Pulmonary Disease
Ottenheijm, Coen A. C., Heunks, Leo M. A., Hafmans, Theo, Benoist, Caroline, Zhou, Honghui, ...
Rationale: Recently, we have shown that Ca2+-activated force generation in diaphragm single fibers is impaired in patients with mild to moderate chronic obstructive pulmonary disease (COPD). For...
Ottenheijm, Coen A. C., Fong, Chi, Vangheluwe, Peter, Wuytack, Frank, Babu, Gopal J., Periasamy, Muthu, ...
Previous work suggested that altered Ca2+ homeostasis might contribute to dysfunction of nebulin-free muscle, as gene expression analysis revealed that the sarco(endo)plasmic reticulum Ca2+-ATPase...