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001-es BibID:	BIBFORM106457
035-os BibID:	(scopus)85144071015 (wos)000895116500001
Első szerző:	Szentesi Péter (élettanász)
Cím:	Disrupted T-tubular network accounts for asynchronous calcium release in MTM1-deficient skeletal muscle / Szentesi Peter, Dienes Beatrix, Kutchukian Candice, Czirjak Tamas, Buj-Bello Anna, Jacquemond Vincent, Csernoch László
Dátum:	2023
ISSN:	0022-3751 1469-7793
Megjegyzések:	In mammalian skeletal muscle, the propagation of surface membrane depolarization into the interior of the muscle fibre along the transverse (T) tubular network is essential for the synchronized release of calcium from the sarcoplasmic reticulum (SR) via ryanodine receptors (RyRs) in response to the conformational change in the voltage-sensor dihydropyridine receptors. Deficiency in 3-phosphoinositide phosphatase myotubularin (MTM1) has been reported to disrupt T-tubules, resulting in impaired SR calcium release. Here confocal calcium transients recorded in muscle fibres of MTM1-deficient mice were compared with the results from a model where propagation of the depolarization along the T-tubules was modelled mathematically with disruptions in the network assumed to modify the access and transmembrane resistance as well as the capacitance. If, in simulations, T-tubules were assumed to be partially or completely inaccessible to the depolarization and RyRs at these points to be prime for calcium-induced calcium release, all the features of measured SR calcium release could be reproduced. We conclude that the inappropriate propagation of the depolarization into the fibre interior is the initial critical cause of severely impaired SR calcium release in MTM1 deficiency, while the Ca2+-triggered opening of RyRs provides an alleviating support to the diseased process.
Tárgyszavak:	Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk calcium release ryanodine receptor sarcoplasmic reticulum MTM1 T-tubule
Megjelenés:	Journal Of Physiology-London. - 601 : 1 (2023), p. 99-121. -
További szerzők:	Dienes Beatrix (1972-) (élettanász, molekuláris biológus) Kutchukian, Candice Czirják Tamás Buj-Bello, Anna Jacquemond, Vincent Csernoch László (1961-) (élettanász)
Pályázati támogatás:	NKFIH K_137600 Egyéb TKP2020-NKA-04 Egyéb 2020?4.1.1-TKP2020 Egyéb
Internet cím:	Szerző által megadott URL DOI Intézményi repozitóriumban (DEA) tárolt változat
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