Results 41 to 50 of about 35,458 (213)

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

Desmin and Cardiac Disease [PDF]

Circulation Research, 2018
Desmin, the intermediate filament protein in striated muscle, is an essential component of the striated muscle cell cytoskeleton. In muscle, the cytoskeleton is a multifunctional, dynamic, and complex structure that serves as a scaffold to maintain the structural integrity and architecture of the cardiomyocyte.
Sonia R, Singh, Jeffrey, Robbins
openaire   +2 more sources

MASH Background Confers Enhanced Disease Susceptibility and Acetaminophen Toxicity in iPSC‐Derived Liver Organoids

Advanced Science, EarlyView.
This work establishes a novel method for generating multicellular liver organoids from control and MASH donor iPSCs. The model recapitulates several disease‐specific characteristics, with MASH donor‐derived organoids showing higher susceptibility. Lipidomic profiling of MASH organoids closely resembles MASH liver biopsies.
Ekta Minocha, Ashwani Kumar Gupta, Nate Schmidt, Richard M. Green, John G. Purdy, Jason A. Wertheim   +5 more
wiley   +1 more source

Desmin enters the nucleus of cardiac stem cells and modulates Nkx2.5 expression by participating in transcription factor complexes that interact with the nkx2.5 gene

Biology Open, 2016
The transcription factor Nkx2.5 and the intermediate filament protein desmin are simultaneously expressed in cardiac progenitor cells during commitment of primitive mesoderm to the cardiomyogenic lineage.
Christiane Fuchs, Sonja Gawlas, Philipp Heher, Sofia Nikouli, Hannah Paar, Mario Ivankovic, Martina Schultheis, Julia Klammer, Teresa Gottschamel, Yassemi Capetanaki, Georg Weitzer   +10 more
doaj   +1 more source

Novel Desmin Mutation Causing Myofibrillar Myopathy in a Hmong Family

Frontiers in Neurology, 2020
Myofibrillar myopathies (MFM) are a clinically and genetically heterogenous group of inherited myopathies characterized by aggregation of Z-disc proteins. Mutations in desmin account for ~7% of MFM.
Stefan Nicolau, Benjamin M. Howe, Elie Naddaf   +2 more
doaj   +1 more source

Desmin expression in rhabdomyosarcoma: influence of the desmin clone and immunohistochemical method. [PDF]

Journal of Clinical Pathology, 1995
AIM--To determine which, if any, of five commercially available desmin clones is most reliable at labelling desmin filaments and whether the enhanced polymer one step (EPOS) method of labelling is of any advantage in the routine diagnostic laboratory. METHODS--Thirty four rhabdomyosarcomas from the files at The Hospital for Sick Children, Great Ormond ...
L, Pollock, D, Rampling, S E, Greenwald, M, Malone   +3 more
openaire   +2 more sources

BIN1 and ALDH1B1 Deficiency in Colonic Smooth Muscle Drives Mitochondrial Dysfunction and Fibrosis in Slow‐Transit Constipation

Advanced Science, EarlyView.
Slow‐transit constipation (STC) is a disabling motility disorder with unclear smooth‐muscle mechanisms. Spatial proteomic analysis of STC patient colon reveals both the central pathogenic role of smooth muscle cells (SMCs) in STC and novel regulators of intestinal motility, BIN1 and ALDH1B1.
Jianbo Liu, Hao Zhang, Wenhao Qiao, Ran Liu, Jie Sun, Xiaopei Li, Qin Li, Dongbo Zhao, Dawei Chen, Jingxin Li, Shuxiao Dong   +10 more
wiley   +1 more source

Treatment of Dystrophic mdx Mice with an ADAMTS-5 Specific Monoclonal Antibody Increases the Ex Vivo Strength of Isolated Fast Twitch Hindlimb Muscles

Biomolecules, 2020
Aberrant extracellular matrix synthesis and remodeling contributes to muscle degeneration and weakness in Duchenne muscular dystrophy (DMD). ADAMTS-5, a secreted metalloproteinase with catalytic activity against versican, is implicated in myogenesis and ...
Alex B. Addinsall, Leonard G. Forgan, Natasha L. McRae, Rhys W. Kelly, Penny L. McDonald, Bryony McNeill, Daniel R. McCulloch, Nicole Stupka   +7 more
doaj   +1 more source

Bioengineered Exosome‐Loaded Immunomodulatory Bioadhesive Spray Reverses Liver Fibrosis and Metabolic Dysfunction That Triggers Beneficial Gut‐Liver Crosstalk in Chronic Fatty Liver Disease

Advanced Science, EarlyView.
Umbilical cord stem cell‐derived exosomes encapsulated in an immunomodulatory bioadhesive hydrogel spray enable localized therapy, sustained vesicle delivery, macrophage polarization, suppress systemic inflammation, and mitigate glucose and insulin homeostasis impairments in MAFLD.
Triya Saha, Ayushi Mairal, Shreya Mehrotra, Shiv K. Sarin, Ashok Kumar   +4 more
wiley   +1 more source

Impaired Autophagic Flux in Skeletal Muscle of Plectin‐Related Epidermolysis Bullosa Simplex With Muscular Dystrophy

Journal of Cachexia, Sarcopenia and Muscle
Background Plectin, a multifunctional cytolinker and intermediate filament stabilizing protein, is essential for muscle fibre integrity and function. Mutations in the human plectin gene (PLEC) cause autosomal recessive epidermolysis bullosa simplex with ...
Michaela M. Zrelski, Margret Eckhard, Petra Fichtinger, Sabrina Hösele, Andy Sombke, Leonid Mill, Monika Kustermann, Wolfgang M. Schmidt, Fiona Norwood, Ursula Schlötzer‐Schrehardt, Gerhard Wiche, Rolf Schröder, Lilli Winter   +12 more
doaj   +1 more source