Journal
JOURNAL OF BIOLOGICAL CHEMISTRY
Volume 284, Issue 27, Pages 18202-18209Publisher
AMER SOC BIOCHEMISTRY MOLECULAR BIOLOGY INC
DOI: 10.1074/jbc.M109.010744
Keywords
-
Categories
Funding
- National Institutes of Health [RO1-DK042394, RO1-HL052173, PO1-HL057346]
- Medical Research Council
- Papworth National Health Service Trust
- Medical Research Council [G0601840, G0500306] Funding Source: researchfish
- MRC [G0500306, G0601840] Funding Source: UKRI
Ask authors/readers for more resources
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-kappa B 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.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available