ER responses play a key role in Swiss-Cheese/Neuropathy Target Esterase-associated neurodegeneration.

ABSTRACT: Swiss Cheese (SWS) is the Drosophila orthologue of Neuropathy Target Esterase (NTE), a phospholipase that when mutated has been shown to cause a spectrum of disorders in humans that range from intellectual disabilities to ataxia. Loss of SWS in Drosophila also causes locomotion deficits, age-dependent neurodegeneration, and an increase in lysophosphatidylcholine (LPC) and phosphatidylcholine (PC). SWS is localized to the Endoplasmic Reticulum (ER), and recently, it has been shown that perturbing the membrane lipid composition of the ER can lead to the activation of ER stress responses through the inhibition of the Sarco/Endoplasmic Reticulum Ca²⁺ ATPase (SERCA). To investigate whether ER stress induction occurs in NTE-associated disorders, we used the fly sws null mutant as a mo

SUBMITTER: Sunderhaus ER 

PROVIDER: S-EPMC6690343 | biostudies-literature | 2019 Oct

REPOSITORIES: biostudies-literature