[HTML][HTML] The endoplasmic reticulum (ER)-associated degradation system regulates aggregation and degradation of mutant neuroserpin
Z Ying, H Wang, H Fan, G Wang - Journal of Biological Chemistry, 2011 - ASBMB
Familial encephalopathy with neuroserpin inclusion bodies is a neurodegenerative disorder
characterized by the accumulation of neuroserpin polymers in the endoplasmic reticulum
(ER) of cortical and subcortical neurons in the CNS because of neuroserpin point mutations.
ER-associated degradation (ERAD) is involved in mutant neuroserpin degradation. In this
study, we demonstrate that two ER-associated E3 ligases, Hrd1 and gp78, are involved in
the ubiquitination and degradation of mutant neuroserpin. Overexpression of Hrd1 and gp78 …
characterized by the accumulation of neuroserpin polymers in the endoplasmic reticulum
(ER) of cortical and subcortical neurons in the CNS because of neuroserpin point mutations.
ER-associated degradation (ERAD) is involved in mutant neuroserpin degradation. In this
study, we demonstrate that two ER-associated E3 ligases, Hrd1 and gp78, are involved in
the ubiquitination and degradation of mutant neuroserpin. Overexpression of Hrd1 and gp78 …