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001-es BibID:BIBFORM100024
035-os BibID:(cikkazonosító)1319 (scopus)85123310479 (wos)000755993200001
Első szerző:Pierantozzi, Enrico
Cím:Impaired Intracellular Ca2+ Dynamics, M-Band and Sarcomere Fragility in Skeletal Muscles of Obscurin KO Mice / Pierantozzi Enrico, Szentesi Péter, Paolini Cecilia, Dienes Beatrix, Fodor János, Oláh Tamás, Colombini Barbara, Rassier Dilson E., Rubino Egidio Maria, Lange Stephan, Rossi Daniela, Csernoch László, Bagni Maria Angela, Reggiani Carlo, Sorrentino Vincenzo
Dátum:2022
ISSN:1661-6596 1422-0067
Megjegyzések:Obscurin is a giant sarcomeric protein expressed in striated muscles known to establish several interactions with other proteins of the sarcomere, but also with proteins of the sarcoplasmic reticulum and costameres. Here, we report experiments aiming to better understand the contribution of obscurin to skeletal muscle fibers, starting with a detailed characterization of the diaphragm muscle function, which we previously reported to be the most affected muscle in obscurin (Obscn) KO mice. Twitch and tetanus tension were not significantly different in the diaphragm of WT and Obscn KO mice, while the time to peak (TTP) and half relaxation time (HRT) were prolonged. Differences in force-frequency and force-velocity relationships and an enhanced fatigability are observed in an Obscn KO diaphragm with respect to WT controls. Voltage clamp experiments show that a sarcoplasmic reticulum's Ca2+ release and SERCA reuptake rates were decreased in muscle fibers from Obscn KO mice, suggesting that an impairment in intracellular Ca2+ dynamics could explain the observed differences in the TTP and HRT in the diaphragm. In partial contrast with previous observations, Obscn KO mice show a normal exercise tolerance, but fiber damage, the altered sarcomere ultrastructure and M-band disarray are still observed after intense exercise.
Tárgyszavak:Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
obscurin
skeletal muscle
calcium dynamics
muscle fiber damage
kinetics of contraction
sarcoplasmic reticulum
exercise
Megjelenés:International Journal Of Molecular Sciences. - 23 : 3 (2022), p. 1319. -
További szerzők:Szentesi Péter (1967-) (élettanász) Paolini, Cecilia Dienes Beatrix (1972-) (élettanász, molekuláris biológus) Fodor János (1973-) (élettanász, biotechnológus) Oláh Tamás (1983-) (élettanász) Colombini, Barbara Rassier, Dilson E. Rubino, Egidio Maria Lange, Stephan Rossi, Daniela Csernoch László (1961-) (élettanász) Bagni, Maria Angela Reggiani, Carlo Sorrentino, Vincenzo
Pályázati támogatás:K 137600
OTKA
TKP2020-NKA-04
Egyéb
2020-4.1.1-TKP2020
Egyéb
Internet cím:Szerző által megadott URL
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2.

001-es BibID:BIBFORM080103
035-os BibID:(cikkazonosító)3361 (scopus)85071306288 (wos)000477041100245
Első szerző:Pierantozzi, Enrico
Cím:Calcium Homeostasis Is Modified in Skeletal Muscle Fibers of Small Ankyrin1 Knockout Mice / Enrico Pierantozzi, Péter Szentesi, Dána Al-Gaadi, Tamás Oláh, Beatrix Dienes, Mónika Sztretye, Daniela Rossi, Vincenzo Sorrentino, László Csernoch
Dátum:2019
ISSN:1661-6596 1422-0067
Megjegyzések:Small Ankyrins (sAnk1) are muscle-specific isoforms generated by the Ank1 gene that participate in the organization of the sarcoplasmic reticulum (SR) of striated muscles. Accordingly, the volume of SR tubules localized around the myofibrils is strongly reduced in skeletal muscle fibers of 4- and 10-month-old sAnk1 knockout (KO) mice, while additional structural alterations only develop with aging. To verify whether the lack of sAnk1 also alters intracellular Ca2+ handling, cytosolic Ca2+ levels were analyzed in stimulated skeletal muscle fibers from 4- and 10-month-old sAnk1 KO mice. The SR Ca2+ content was reduced in sAnk1 KO mice regardless of age. The amplitude of the Ca2+ transients induced by depolarizing pulses was decreased in myofibers of sAnk1 KO with respect to wild type (WT) fibers, while their voltage dependence was not affected. Furthermore, analysis of spontaneous Ca2+ release events (sparks) on saponin-permeabilized muscle fibers indicated that the frequency of sparks was significantly lower in fibers from 4-month-old KO mice compared to WT. Furthermore, both the amplitude and spatial spread of sparks were significantly smaller in muscle fibers from both 4- and 10-month-old KO mice compared to WT. These data suggest that the absence of sAnk1 results in an impairment of SR Ca2+ release, likely as a consequence of a decreased Ca2+ store due to the reduction of the SR volume in sAnk1 KO muscle fibers.
Tárgyszavak:Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
sarcoplasmic reticulum
sAnk1
calcium release
spark
Megjelenés:International Journal of Molecular Sciences. - 20 : 13 (2019), p. 3361. -
További szerzők:Szentesi Péter (1967-) (élettanász) Al-Gaadi, Dana (1991-) Oláh Tamás (1983-) (élettanász) Dienes Beatrix (1972-) (élettanász, molekuláris biológus) Sztretye Mónika (1981-) (élettanász, elektrofiziológus) Rossi, Daniela Sorrentino, Vincenzo Csernoch László (1961-) (élettanász)
Pályázati támogatás:NKFIH K-115461
NKFIH
GINOP-2.3.2-15-2016-00040
GINOP
EFOP-3.6.2-16-2017-00006
EFOP
ÚNKP-18-4-DE-157
Egyéb
Internet cím:Szerző által megadott URL
DOI
Intézményi repozitóriumban (DEA) tárolt változat
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