CLN8p deficiency impairs lysosomal motility and protein distribution in hippocampal neuronal model

PESAOLA F; NOHER DE HALAC I; BISBAL M

Buenos Aires

Congreso; Alexander von Humboldt Colloquium Argentina; 2018

Alexander von Humboldt-Stiftung/Foundation

CLN8 protein (CLN8p) is an Endoplasmic Reticulum (ER)-residenttransmembrane protein that shuttles between ER and Golgi Apparatus,whose malfunction triggers the CLN8 disease. Although Its role is still poorlyknown, it was related to different cellular pathways, such as lipidmetabolism, autophagy, apoptosis and vesicular/membrane traffic.Moreover, how mutations of an ER-resident protein cause lysosomal storageremain unknown. In this project we aim to study the effects of CLN8pdeficiency on lysosomal dynamics and protein distribution in neuronalmodel.