Article
Rab1-dependent ER-Golgi transport dysfunction is a common pathogenic mechanism in SOD1, TDP-43 and FUS-associated ALS.
Acta neuropathologica - 1 Nov 2015
Soo Kai Y, Halloran Mark, Sundaramoorthy Vinod, Parakh Sonam, Toth Reka P, Southam Katherine A, McLean Catriona A, Lock Peter, King Anna, Farg Manal A, Atkin Julie D
Abstract excerpt
Several diverse proteins are linked genetically/pathologically to neurodegeneration in amyotrophic lateral sclerosis (ALS) including SOD1, TDP-43 and FUS. Using a variety of cellular and biochemical techniques, we demonstrate that ALS-associated mutant TDP-43, FUS and SOD1 inhibit protein transport between the endoplasmic reticulum (ER) and Golgi apparatus in neuronal cells. ER-Golgi transport was also inhibited...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.