Endoplasmic reticulum-associated degradation (ERAD) and autophagy cooperate to degrade polymerogenic mutant serpins.

@article{Kroeger2009EndoplasmicRD,
  title={Endoplasmic reticulum-associated degradation (ERAD) and autophagy cooperate to degrade polymerogenic mutant serpins.},
  author={Heike Kroeger and Elena Miranda and Ian MacLeod and Juan P{\'e}rez and Damian C Crowther and Stefan J Marciniak and David A. Lomas},
  journal={The Journal of biological chemistry},
  year={2009},
  volume={284 34},
  pages={22793-802}
}
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 alpha(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… CONTINUE READING

Citations

Publications citing this paper.
Showing 1-10 of 48 extracted citations

Aggrephagy: Selective Disposal of Protein Aggregates by Macroautophagy

International journal of cell biology • 2012
View 3 Excerpts
Highly Influenced

References

Publications referenced by this paper.
Showing 1-2 of 2 references

Mutant Serpin Degradation 22802 JOURNAL OF BIOLOGICAL CHEMISTRY VOLUME

C. Yun, A. Stanhill, +7 authors D. Ron
Proc. Natl. Acad. Sci. U.S.A • 2008
View 1 Excerpt

NUMBER 34 JOURNAL OF BIOLOGICAL CHEMISTRY 22801 by gest on N ovem er 12

S. J. Marciniak, D. Ron
Physiol. Rev • 2006
View 2 Excerpts

Similar Papers

Loading similar papers…