[HTML][HTML] Endoplasmic reticulum-associated degradation (ERAD) and autophagy cooperate to degrade polymerogenic mutant serpins
The serpinopathies are a family of diseases characterized by the accumulation of ordered
polymers of mutant protein within the endoplasmic reticulum. They are a diverse group
including α 1-antitrypsin deficiency and the inherited dementia familial encephalopathy with
neuroserpin inclusion bodies or FENIB. We have used transient transfection of COS7 cells
and mouse embryonic fibroblasts, PC12 cell lines that conditionally express wild type and
mutant neuroserpin and fly models of FENIB to assess the cellular handling of wild type and …
polymers of mutant protein within the endoplasmic reticulum. They are a diverse group
including α 1-antitrypsin deficiency and the inherited dementia familial encephalopathy with
neuroserpin inclusion bodies or FENIB. We have used transient transfection of COS7 cells
and mouse embryonic fibroblasts, PC12 cell lines that conditionally express wild type and
mutant neuroserpin and fly models of FENIB to assess the cellular handling of wild type and …