Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms
Identifieur interne : 000A27 ( Main/Curation ); précédent : 000A26; suivant : 000A28Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms
Auteurs : Leslie I. Grad [Canada] ; Justin J. Yerbury [Australie] ; Bradley J. Turner ; William C. Guest [Canada] ; Edward Pokrishevsky [Canada] ; Megan A. O Eill [Canada] ; Anat Yanai [Canada] ; Judith M. Silverman [Canada] ; Rafaa Zeineddine [Australie] ; Lisa Corcoran [Australie] ; Janet R. Kumita [Royaume-Uni] ; Leila M. Luheshi [Royaume-Uni] ; Masoud Yousefi [Canada] ; Bradley M. Coleman [Australie] ; Andrew F. Hill [Australie] ; Steven S. Plotkin [Canada] ; Ian R. Mackenzie [Canada] ; Neil R. Cashman [Canada]Source :
- Proceedings of the National Academy of Sciences of the United States of America [ 0027-8424 ] ; 2014.
Abstract
Amyotrophic lateral sclerosis (ALS), an incurable motor neuron disease, is associated with mutation and misfolding of the Cu/Zn superoxide dismutase (SOD1) protein. Prior studies found that mutant misfolded SOD1 can convert wild-type (WT) SOD1 to a misfolded form inside living cells in a prion-like fashion. We now report that misfolded WT SOD1 can be transmitted from cell to cell, and that propagated protein misfolding can be perpetuated. Misfolded SOD1 transmission between cells can be mediated through release and uptake of protein aggregates or via small membrane-bounded transport vesicles called exosomes. These mechanisms may help explain why sporadic ALS, without a known genetic cause, can spread systematically from region to region in a progressive manner.
Url:
DOI: 10.1073/pnas.1312245111
PubMed: 24550511
PubMed Central: 3948312
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PMC:3948312Le document en format XML
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<front><div type="abstract" xml:lang="en"><title>Significance</title>
<p>Amyotrophic lateral sclerosis (ALS), an incurable motor neuron disease, is associated with mutation and misfolding of the Cu/Zn superoxide dismutase (SOD1) protein. Prior studies found that mutant misfolded SOD1 can convert wild-type (WT) SOD1 to a misfolded form inside living cells in a prion-like fashion. We now report that misfolded WT SOD1 can be transmitted from cell to cell, and that propagated protein misfolding can be perpetuated. Misfolded SOD1 transmission between cells can be mediated through release and uptake of protein aggregates or via small membrane-bounded transport vesicles called exosomes. These mechanisms may help explain why sporadic ALS, without a known genetic cause, can spread systematically from region to region in a progressive manner.</p>
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