Dysfunction of the ubiquitin-proteasome system has recently been implicated in the pathogenesis of some untreatable myodegenerative diseases characterized by the formation of ubiquitinated inclusions in skeletal muscles. We have developed an in vitro model of proteasomal dysfunction by applying inhibitors of the proteasome to primary adult human skeletal muscle cultures. Our data show that proteasome inhibition causes both cytoplasmic accumulation of ubiquitinated inclusions and apoptotic death, the latter through accumulation of active caspase-3.
Apoptosis induced by proteasome inhibition in human myoblast cultures / J. Sassone, A. Ciammola, C. Tiloca, M. Glionna, G. Meola, E. Mancinelli, V. Silani. - In: EUROPEAN JOURNAL OF HISTOCHEMISTRY. - ISSN 1121-760X. - 50:2(2006), pp. 109-118.
Apoptosis induced by proteasome inhibition in human myoblast cultures
J. Sassone;G. Meola;E. MancinelliPenultimo
;V. SilaniUltimo
2006
Abstract
Dysfunction of the ubiquitin-proteasome system has recently been implicated in the pathogenesis of some untreatable myodegenerative diseases characterized by the formation of ubiquitinated inclusions in skeletal muscles. We have developed an in vitro model of proteasomal dysfunction by applying inhibitors of the proteasome to primary adult human skeletal muscle cultures. Our data show that proteasome inhibition causes both cytoplasmic accumulation of ubiquitinated inclusions and apoptotic death, the latter through accumulation of active caspase-3.
Pubblicazioni consigliate
I metadati presenti in AIR sono rilasciati con licenza CCO mentre i file delle pubblicazioni sono rilasciati con licenza CC BY-SA, salvo diversa indicazione. In caso di violazione di copyright, contattare l'assistenza di AIR
