Stress of endoplasmic reticulum and expression of VAPB and Rab1 in cultured motor neurons from a mouse model of Amyotrophic Lateral Sclerosis

Abstract

Endoplasmic reticulum is a multifunctional organelle involved in protein synthesis, processing, and modeling, as well as participates of intracellular traffic and calcium signaling. VAPB is an endoplasmic reticulum transnsmembrane protein that interacts with mitochondria during calcium homeostasis, participates of cellular signalling, cytoarchitecture, unfolded protein response and traffic between endoplasmic reticulum and golgi apparatus. This trafficking is also performed by Rab1. Endoplasmic reticulum stress might be associated to dysfunction of VAPB and Rab1, and it is common to many neurodegenerative diseases such as amyotrophic lateral sclerosis. In view of this, this study aims to investigate the association among the expression of VAPB, Rab1, and endoplasmic reticulum stress in cultured motor neurons of transgenic mice expressing mutant SOD1 (G93A), non-mutant SOD1, or control mice B6SJL. The methodology will employ a genotyping, culture of motor neurons, immunocytochemistry, western blot, and exogenous expression of Rab1.