Results 1 to 10 of about 18,110 (202)
Desmin variants: Trigger for cardiac arrhythmias?
Frontiers in Cell and Developmental Biology, 2022 Desmin (DES) is a classical type III intermediate filament protein encoded by the DES gene. Desmin is abundantly expressed in cardiac, skeletal, and smooth muscle cells.
Wei Su +5 more
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Desmin accumulation restrictive cardiomyopathy and atrioventricular block associated with desmin gene defects [PDF]
European Journal of Heart Failure, 2006 Primary desminopathies are caused by desmin gene [DES (MIM*125660)] mutations. The clinical spectrum includes pure myopathies, cardiomuscular diseases and cardiomyopathies. Patients with restrictive cardiomyopathy (RCM) plus atrioventricular block (AVB) due to DES defects are frequently unrecognized unless desmin accumulation is specifically ...
Eloisa Arbustini +2 more
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Desmin’s conformational modulation by hydrophobicity
Türk Biyokimya DergisiNucleocytoplasmic transport is one of the key features in regulation of cellular physiology. Developing a better understanding of the molecular mechanism underlying the nucleocytoplasmic shuttling of proteins can broaden our perspective and understanding
Kural Mangıt Ecem +2 more
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Antioxidants, 2023 The type III intermediate filament proteins vimentin and GFAP are modulated by oxidants and electrophiles, mainly through perturbation of their single cysteine residues.
Diego Moneo-Corcuera +3 more
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Brain, 2004 Desmin myopathy is a recently identified disease associated with mutations in desmin or alphaB-crystallin. Typically, the illness presents with lower limb muscle weakness slowly spreading to involve truncal, neck-flexor, facial, bulbar and respiratory muscles.
L G, Goldfarb +3 more
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Scientific Reports, 2022 Desmin is the guardian of striated muscle integrity, permitting the maintenance of muscle shape and the efficiency of contractile activity. It is also a key mediator of cell homeostasis and survival.
Charlotte Claeyssen +2 more
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Toxins, 2022 Carboxylic ionophores, such as monensin, salinomycin and lasalocid, are polyether antibiotics used widely in production animals for the control of coccidiosis, as well as for the promotion of growth and feed efficiency.
Danielle Henn +3 more
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Multiple sites in αB-crystallin modulate its interactions with desmin filaments assembled in vitro. [PDF]
PLoS ONE, 2011 The β3- and β8-strands and C-terminal residues 155-165 of αB-crystallin were identified by pin arrays as interaction sites for various client proteins including the intermediate filament protein desmin.
Scott A Houck +3 more
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Congenital sacrococcygeal rhabdomyosarcoma
African Journal of Paediatric Surgery, 2022 Rhabdomyosarcoma (RMS) is one of the common malignant soft-tissue sarcomas affecting children. It originates from the embryonic mesenchyme precursor of striated muscle and is frequently seen in the head-and-neck region, genitourinary system and ...
Renuka Venkata Inuganti +3 more
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Drosophila Nesprin-1 Isoforms Differentially Contribute to Muscle Function
Cells, 2021 Nesprin-1 is a large scaffold protein connecting nuclei to the actin cytoskeleton via its KASH and Calponin Homology domains, respectively. Nesprin-1 disconnection from nuclei results in altered muscle function and myonuclei mispositioning.
Alexandre Rey +3 more
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