[HTML][HTML] Degradation of trafficking-defective long QT syndrome type II mutant channels by the ubiquitin-proteasome pathway
Q Gong, DR Keeney, M Molinari, Z Zhou - Journal of Biological Chemistry, 2005 - ASBMB
Mutations in the human ether-a-go-go-related gene (hERG) cause chromosome 7-linked
long QT syndrome type II (LQT2). We have shown previously that LQT2 mutations lead to
endoplasmic reticulum (ER) retention and rapid degradation of mutant hERG proteins. In this
study we examined the role of the ubiquitin-proteasome pathway in the degradation of the
LQT2 mutation Y611H. We showed that proteasome inhibitors N-acetyl-l-leucyl-l-leucyl-l-
norleucinal and lactacystin but not lysosome inhibitor leupeptin inhibited the degradation of …
long QT syndrome type II (LQT2). We have shown previously that LQT2 mutations lead to
endoplasmic reticulum (ER) retention and rapid degradation of mutant hERG proteins. In this
study we examined the role of the ubiquitin-proteasome pathway in the degradation of the
LQT2 mutation Y611H. We showed that proteasome inhibitors N-acetyl-l-leucyl-l-leucyl-l-
norleucinal and lactacystin but not lysosome inhibitor leupeptin inhibited the degradation of …