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001-es BibID:	BIBFORM078604
035-os BibID:	(WOS)000469204700012 (Scopus)85064219867
Első szerző:	Kutchukian, Candice
Cím:	Ca2+-induced sarcoplasmic reticulum Ca2+ release in myotubularin-deficient muscle fibers / Candice Kutchukian, Peter Szentesi, Bruno Allard, Anna Buj-Bello, Laszlo Csernoch, Vincent Jacquemond
Dátum:	2019
ISSN:	0143-4160
Megjegyzések:	Skeletal muscle deficiency in the 3-phosphoinositide (PtdInsP) phosphatase myotubularin (MTM1) causes myotubular myopathy which is associated with severe depression of voltage-activated sarcoplasmic reticulum Ca2+ release through ryanodine receptors. In the present study we aimed at further understanding how Ca2+ release is altered in MTM1-deficient muscle fibers, at rest and during activation. While in wild-type muscle fibers, SR Ca2+ release exhibits fast stereotyped kinetics of activation and decay throughout the voltage range of activation, Ca2+ release in MTM1-deficient muscle fibers exhibits slow and unconventional kinetics at intermediate voltages, suggestive of partial loss of the normal control of ryanodine receptor Ca2+ channel activity. In addition, the diseased muscle fibers at rest exhibit spontaneous elementary Ca2+ release events at a frequency 30 times greater than that of control fibers. Eighty percent of the events have spatiotemporal properties of archetypal Ca2+ sparks while the rest take either the form of lower amplitude, longer duration Ca2+ release events or of a combination thereof. The events occur at preferred locations in the fibers, indicating spatially uneven distribution of the parameters determining spontaneous ryanodine receptor 1 opening. Spatially large Ca2+ release sources were obviously involved in some of these events, suggesting that opening of ryanodine receptors in one cluster can activate opening of ryanodine receptors in a neighboring one. Overall results demonstrate that opening of Ca2+-activated ryanodine receptors is promoted both at rest and during excitation-contraction coupling in MTM1-deficient muscle fibers. Because access to this activation mode is denied to ryanodine receptors in healthy skeletal muscle, this may play an important role in the associated disease situation.
Tárgyszavak:	Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk Skeletal muscle Ryanodine receptor Sarcoplasmic reticulum Ca2+ release Myotubular myopathy
Megjelenés:	Cell Calcium. - 80 (2019), p. 91-100. -
További szerzők:	Szentesi Péter (1967-) (élettanász) Allard, Bruno Buj-Bello, Anna Csernoch László (1961-) (élettanász) Jacquemond, Vincent
Internet cím:	Szerző által megadott URL DOI Intézményi repozitóriumban (DEA) tárolt változat
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