Results 51 to 60 of about 43,204 (229)

Cytoskeleton–Membrane Uncoupling in Duchenne Muscular Dystrophy: Implications for Newborn Screening and Early Protection

Cytoskeleton, EarlyView.
ABSTRACT The cytoskeleton of striated muscle integrates force transmission, mechanotransduction, and sarcolemmal stability through coordinated networks of sarcomeres, costameres, and intermediate filaments. Together, these systems establish mechanical continuity between the contractile apparatus, the sarcolemma, and the extracellular matrix.
Houda Cohen, Nora Hosny, Brian R. Thompson, Joseph M. Metzger   +3 more
wiley   +1 more source

Developmental Alterations in Heart Biomechanics and Skeletal Muscle Function in Desmin Mutants Suggest an Early Pathological Root for Desminopathies

Cell Reports, 2015
Desminopathies belong to a family of muscle disorders called myofibrillar myopathies that are caused by Desmin mutations and lead to protein aggregates in muscle fibers.
Caroline Ramspacher, Emily Steed, Francesco Boselli, Rita Ferreira, Nathalie Faggianelli, Stéphane Roth, Coralie Spiegelhalter, Nadia Messaddeq, Le Trinh, Michael Liebling, Nikhil Chacko, Federico Tessadori, Jeroen Bakkers, Jocelyn Laporte, Karim Hnia, Julien Vermot   +15 more
doaj   +1 more source

Interference of amino-terminal desmin fragments with desmin filament formation

, 2009
Short polypeptides from intermediate filament (IF) proteins containing one of the two IF-consensus motifs interfere severely with filament assembly in vitro.
Kleiner, Helga, Mücke, Norbert, Zentgraf, Hanswalter, Bär, Harald, Katus, Hugo A., Aebi, Ueli, Herrmann, Harald, Sharma, Sarika   +7 more
core   +1 more source

Assembly and Maintenance of Myofibrils in the Heart

Cytoskeleton, EarlyView.
ABSTRACT The assembly of actin and myosin to the paracrystalline arrangement of myofibrils in striated muscle, that is, heart and skeletal, has puzzled many a cell biologist. Different models, based on cultured cardiomyocytes, were proposed for the last 30 years to explain the sequence of events.
Elisabeth Ehler
wiley   +1 more source

Desmin mutations in the terminal consensus motif prevent synemin-desmin heteropolymer filament assembly

, 2011
Disorganization of the desmin network is associated with cardiac and skeletal myopathies characterized by accumulation of desmin-containing aggregates in the cells.
Agbulut O., Zhigang Xue, Xue Z., Chourbagi O., Vicart P., Denise Paulin, Patrick Vicart, Oussama Chourbagi, Paulin D., Marianna Carinci, Carinci M., Francine Bruston, Bruston F., Onnik Agbulut   +13 more
core   +1 more source

Challenges and Pitfalls to Diagnosing NUTM1‐Rearranged Neoplasia of the Pancreas by Cytology and Ancillary Studies

Diagnostic Cytopathology, EarlyView.
ABSTRACT Fine‐needle aspiration cytology specimens are frequently utilized for ancillary studies to identify diagnostic and prognostic information. This case highlights diagnostic pitfalls and challenges in diagnosing NUTM1‐rearranged neoplasia on pancreatic cytology.
Terrance J. Lynn
wiley   +1 more source

Early signs of architectural and biomechanical failure in isolated myofibers and immortalized myoblasts from desmin-mutant knock-in mice

Scientific Reports, 2017
In striated muscle, desmin intermediate filaments interlink the contractile myofibrillar apparatus with mitochondria, nuclei, and the sarcolemma. The desmin network’s pivotal role in myocytes is evident since mutations in the human desmin gene cause ...
Stefanie Diermeier, Julian Iberl, Kristina Vetter, Michael Haug, Charlotte Pollmann, Barbara Reischl, Andreas Buttgereit, Sebastian Schürmann, Marina Spörrer, Wolfgang H. Goldmann, Ben Fabry, Fatiha Elhamine, Robert Stehle, Gabriele Pfitzer, Lilli Winter, Christoph S. Clemen, Harald Herrmann, Rolf Schröder, Oliver Friedrich   +18 more
doaj   +1 more source

Changes in cell shape and desmin intermediate filament distribution are associated with down-regulation of desmin expression in C2C12 myoblasts grown in the absence of extracellular Ca2+

Brazilian Journal of Medical and Biological Research, 2005
Desmin is the main intermediate filament (IF) protein of muscle cells. In skeletal muscle, desmin IFs form a scaffold that interconnects the entire contractile apparatus with the subsarcolemmal cytoskeleton and cytoplasmic organelles.
C.S. Mermelstein, L.M. Amaral, M.I.L. Rebello, J.S.N. Reis, R. Borojevic, M.L. Costa   +5 more
doaj   +1 more source

New roles for desmin in the maintenance of muscle homeostasis

, 2021
Desmin is the primary intermediate filament (IF) of cardiac, skeletal, and smooth muscle. By linking the contractile myofibrils to the sarcolemma and cellular organelles, desmin IF contributes to muscle structural and cellular integrity, force ...
Giulio Agnetti, Shenhav Cohen, Harald Herrmann, Herrmann, Harald, Cohen, Shenhav, Agnetti, Giulio   +5 more
core   +1 more source

Primary Pulmonary Mesenchymal Neoplasm With EWSR1::CREM Fusion: Cytologic Findings and Molecular Diagnosis

Diagnostic Cytopathology, EarlyView.
ABSTRACT Background Primary pulmonary mesenchymal neoplasms with EWSR1::CREM fusion are rare. These lesions are challenging to diagnose by morphology and immunohistochemistry alone. Case A 66‐year‐old woman (ex‐smoker) was found to have a 1.3‐cm right lower lobe lung nodule that had grown very slowly over a 9‐year period.
Priya Upadhyay, Sanjay Mukhopadhyay, Swikrity U. Baskota   +2 more
wiley   +1 more source