Analysis of HeLa cells expressing the G392E mutant of neuroserpin. Accumulation of mutant neuroserpin polymers in the ER is associated with familial encephalopathy with neuroserpin inclusion bodies. Results provide insight into pathways initiated by the polymerization of neuroserpin within the ER.
Roussel BD, Newton TM, Malzer E, Simecek N et al. Sterol metabolism regulates neuroserpin polymer degradation in the absence of the unfolded protein response in the dementia FENIB. Hum Mol Genet 2013 Nov 15;22(22):4616-26. PMID: 23814041
