This review outlines the basic properties of T-tubules in skeletal muscle cells, and the factors that govern reversible vacuolation in T-tubules under experimental conditions. Comparable membranous transformations, involving the plasma membrane or occurring intracellularly, in non-muscle cells are then considered. Finally, the mechanisms of similar transformations in various model membrane systems are discussed. In view of the similarities between reversible vacuolation in the T-system and membrane transformations occurring in a variety of non-muscle cells, it is suggested that reversible vacuolation in T-tubules may be regarded as a general model for tubular-vesicular transformations in biomembranes.
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http://dx.doi.org/10.1006/cbir.2002.0945 | DOI Listing |
Cell Tissue Res
September 2021
Center of Basic Research, Biomedical Research Foundation Academy of Athens, 11527, Athens, Greece.
The desmin-associated protein myospryn, encoded by the cardiomyopathy-associated gene 5 (CMYA5), is a TRIM-like protein associated to the BLOC-1 (Biogenesis of Lysosomes Related Organelles Complex 1) protein dysbindin. Human myospryn mutations are linked to both cardiomyopathy and schizophrenia; however, there is no evidence of a direct causative link of myospryn to these diseases. Therefore, we sought to unveil the role of myospryn in heart and brain.
View Article and Find Full Text PDFJ Neuropathol Exp Neurol
December 2020
Department of Neurology, Faculty of Medicine, University of Yamanashi, Chuou-city, Yamanashi, Japan.
We investigated the immunohistochemical localization of several proteins related to excitation-contraction coupling and ultrastructural alterations of the sarcotubular system in biopsied muscles from a father and a daughter in a family with permanent myopathy with hypokalemic periodic paralysis (PMPP) due to a mutation in calcium channel CACNA1S; p. R1239H hetero. Immunostaining for L-type calcium channels (LCaC) showed linear hyper-stained regions indicating proliferation of longitudinal t-tubules.
View Article and Find Full Text PDFAm J Physiol Heart Circ Physiol
August 2020
Department of Molecular and Integrative Physiology, University of Michigan, Ann Arbor, Michigan.
Cardiac t tubules undergo significant remodeling in various pathological and experimental conditions, which can be associated with mechanical or osmotic stress. In particular, it has been shown that removal of hyposmotic stress can lead to sealing of t tubules. However, the mechanisms underlying the sealing process remain essentially unknown.
View Article and Find Full Text PDFAging Clin Exp Res
February 2009
Institute of Anatomy, Department of Medicine, University of Leipzig, Leipzig, Germany.
Background And Aims: Acute hypoxia is a threatening clinical case of emergency and may result in ultrastructural damage, with complete loss of cellular and organ functions. However, little is known about the differences in hypoxia tolerance between young and old myocardia and the protective effects of radical scavenging agents in acute hypoxic stress situations.
Methods: We investigated the age-dependent differences of the myocardial ultrastructure and antioxidative status (superoxide-dismutase (SOD) activity and malondialdehyde (MDA) content) of young (6 months) and old (22-24 months) Wistar rats (Crl (Wi)Br) after acute respiratory hypoxia of 20 min at 5% v/v O2 in N2O mixture, and the protective effect of Ginkgo biloba extract (EGb 761).
Hum Mol Genet
July 2008
Department of Neurobiology and Genetics, , INSERM U596, CNRS UMR 7104, Université Louis Pasteur de Strasbourg, Collège de France, 67404 Illkirch, France.
Myotubular myopathy (XLMTM, OMIM 310400) is a severe congenital muscular disease due to mutations in the myotubularin gene (MTM1) and characterized by the presence of small myofibers with frequent occurrence of central nuclei. Myotubularin is a ubiquitously expressed phosphoinositide phosphatase with a muscle-specific role in man and mouse that is poorly understood. No specific treatment exists to date for patients with myotubular myopathy.
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