The Role of BiP and the IRE1α-XBP1 Axis in Rhabdomyosarcoma Pathology.
Mahmoud AghaeiAhmad NasimianMarveh RahmatiPhilip KawalecFilip MachajJakub RosikBhavya BhushanS Zahra BathaieNegar AzarpiraMarek Jan ŁosAfshin SamaliDavid PerrinJoseph W GordonSaeid GhavmiPublished in: Cancers (2021)
In summary, this study demonstrates that the overall UPR is upregulated and, more specifically, that the IRE1/sXBP1 axis is active in RMS. The subtype and stage-specific dependency on the UPR machinery in RMS may open new avenues for the development of novel targeted therapeutic strategies and the identification of specific tumor markers in this rare but deadly childhood and young-adult disease.