Calcium signalling in mammalian cell lines expressing wild type and mutant human α1-Antitrypsin.

ABSTRACT: A possible role for calcium signalling in the autosomal dominant form of dementia, familial encephalopathy with neuroserpin inclusion bodies (FENIB), has been proposed, which may point towards a mechanism by which cells could sense and respond to the accumulation of mutant serpin polymers in the endoplasmic reticulum (ER). We therefore explored possible defects in Ca²⁺-signalling, which may contribute to the pathology associated with another serpinopathy, α₁-antitrypsin (AAT) deficiency. Using CHO K1 cell lines stably expressing a wild type human AAT (MAAT) and a disease-causing polymer-forming variant (ZAAT) and the truncated variant (NHK AAT), we measured basal intracellular free Ca²⁺, its responses to thapsigargin (TG), an ER Ca²⁺-ATPase block

SUBMITTER: Malintan NT 

PROVIDER: S-EPMC6872872 | biostudies-literature | 2019 Nov

REPOSITORIES: biostudies-literature