Ontology highlight
ABSTRACT:
SUBMITTER: Grad LI
PROVIDER: S-EPMC3948312 | biostudies-literature | 2014 Mar
REPOSITORIES: biostudies-literature
Grad Leslie I LI Yerbury Justin J JJ Turner Bradley J BJ Guest William C WC Pokrishevsky Edward E O'Neill Megan A MA Yanai Anat A Silverman Judith M JM Zeineddine Rafaa R Corcoran Lisa L Kumita Janet R JR Luheshi Leila M LM Yousefi Masoud M Coleman Bradley M BM Hill Andrew F AF Plotkin Steven S SS Mackenzie Ian R IR Cashman Neil R NR
Proceedings of the National Academy of Sciences of the United States of America 20140218 9
Amyotrophic lateral sclerosis (ALS) is predominantly sporadic, but associated with heritable genetic mutations in 5-10% of cases, including those in Cu/Zn superoxide dismutase (SOD1). We previously showed that misfolding of SOD1 can be transmitted to endogenous human wild-type SOD1 (HuWtSOD1) in an intracellular compartment. Using NSC-34 motor neuron-like cells, we now demonstrate that misfolded mutant and HuWtSOD1 can traverse between cells via two nonexclusive mechanisms: protein aggregates re ...[more]