Official websites use .gov
A
.gov website belongs to an official
government organization in the United States.
Secure .gov websites use HTTPS
A lock (
) or https:// means you've safely
connected to the .gov website. Share sensitive
information only on official, secure websites.
Model of BTZ effect on CAPN3-deficient muscle fibers. CAPN3 deficiency induces increased ubiquitination and degradation of SERCA through the ubiquitin proteasome pathway (UPS). Bortezomib inhibits proteasome activity and thus degradation of SERCA proteins. However, BTZ also strongly inhibits SERCA1 expression at the mRNA level, so that BTZ rescue is mostly observed on SERCA2 proteins. On the other hand, BTZ is able to rescue mutant CAPN3 protein levels carrying missense mutations.
