Neuroserpin polymers activate NF-kappaB by a calcium signaling pathway that is independent of the unfolded protein response.: Neuroserpin polymers and the ER overload response

Mark Davies 1 Elena Miranda Benoit Roussel 1 Randal Kaufman 2 Stefan Marciniak 1 David Lomas 1, *
* Auteur correspondant
1 Department of Medicine
2 Departments of Biological Chemistry and Internal Medicine and the HHMI
Abstract : The autosomal dominant dementia familial encephalopathy with neuroserpin inclusion bodies is characterized by the accumulation of ordered polymers of mutant neuroserpin within the endoplasmic reticulum of neurones. We show here that intracellular neuroserpin polymers activate NF-kappaB by a pathway that is independent of the IRE1, ATF6, and PERK limbs of the canonical unfolded protein response but is dependent on intracellular calcium. This pathway provides a mechanism for cells to sense and react to the accumulation of folded structures of mutant serpins within the endoplasmic reticulum. Our results provide strong support for the endoplasmic reticulum overload response being independent of the unfolded protein response.
Keywords : ER stress EOR serpin UPR serpinopathies conformational diseases
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Article dans une revue
Journal of Biological Chemistry, American Society for Biochemistry and Molecular Biology, 2009, 284 (27), pp.18202-9. 〈10.1074/jbc.M109.010744〉
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Mark Davies, Elena Miranda, Benoit Roussel, Randal Kaufman, Stefan Marciniak, et al.. Neuroserpin polymers activate NF-kappaB by a calcium signaling pathway that is independent of the unfolded protein response.: Neuroserpin polymers and the ER overload response . Journal of Biological Chemistry, American Society for Biochemistry and Molecular Biology, 2009, 284 (27), pp.18202-9. 〈10.1074/jbc.M109.010744〉. 〈inserm-01296769〉

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