naa a22 4500 510746446 CHVBK 20180411083048.0 cr unu---uuuuu 180411e20130301xx s 000 0 eng 10.1007/s12192-012-0371-1 doi (NATIONALLICENCE)springer-10.1007/s12192-012-0371-1 The small heat shock proteins αB-crystallin and Hsp27 suppress SOD1 aggregation in vitro [Elektronische Daten] [Justin Yerbury, Dane Gower, Laura Vanags, Kate Roberts, Jodi Lee, Heath Ecroyd] Amyotrophic lateral sclerosis is a devastating neurodegenerative disease. The mechanism that underlies amyotrophic lateral sclerosis (ALS) pathology remains unclear, but protein inclusions are associated with all forms of the disease. Apart from pathogenic proteins, such as TDP-43 and SOD1, other proteins are associated with ALS inclusions including small heat shock proteins. However, whether small heat shock proteins have a direct effect on SOD1 aggregation remains unknown. In this study, we have examined the ability of small heat shock proteins αB-crystallin and Hsp27 to inhibit the aggregation of SOD1 in vitro. We show that these chaperone proteins suppress the increase in thioflavin T fluorescence associated with SOD1 aggregation, primarily through inhibiting aggregate growth, not the lag phase in which nuclei are formed. αB-crystallin forms high molecular mass complexes with SOD1 and binds directly to SOD1 aggregates. Our data are consistent with an overload of proteostasis systems being associated with pathology in ALS. Cell Stress Society International, 2012 Amyotrophic lateral sclerosis nationallicence SOD1 nationallicence Protein aggregation nationallicence Small heat shock proteins nationallicence Molecular chaperones nationallicence αB-crystallin nationallicence Yerbury Justin Illawarra Health and Medical Research Institute, School of Biological Sciences, University of Wollongong, Northfields Avenue, 2522, Wollongong, NSW, Australia aut Gower Dane Illawarra Health and Medical Research Institute, School of Biological Sciences, University of Wollongong, Northfields Avenue, 2522, Wollongong, NSW, Australia aut Vanags Laura Illawarra Health and Medical Research Institute, School of Biological Sciences, University of Wollongong, Northfields Avenue, 2522, Wollongong, NSW, Australia aut Roberts Kate Illawarra Health and Medical Research Institute, School of Biological Sciences, University of Wollongong, Northfields Avenue, 2522, Wollongong, NSW, Australia aut Lee Jodi Illawarra Health and Medical Research Institute, School of Biological Sciences, University of Wollongong, Northfields Avenue, 2522, Wollongong, NSW, Australia aut Ecroyd Heath Illawarra Health and Medical Research Institute, School of Biological Sciences, University of Wollongong, Northfields Avenue, 2522, Wollongong, NSW, Australia aut Cell Stress and Chaperones Springer Netherlands 18/2(2013-03-01), 251-257 1355-8145 18:2<251 2013 18 12192 https://doi.org/10.1007/s12192-012-0371-1 text/html Onlinezugriff via DOI 1 brief-communication jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s12192-012-0371-1 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Yerbury Justin Illawarra Health and Medical Research Institute, School of Biological Sciences, University of Wollongong, Northfields Avenue, 2522, Wollongong, NSW, Australia aut NATIONALLICENCE 700 1- Gower Dane Illawarra Health and Medical Research Institute, School of Biological Sciences, University of Wollongong, Northfields Avenue, 2522, Wollongong, NSW, Australia aut NATIONALLICENCE 700 1- Vanags Laura Illawarra Health and Medical Research Institute, School of Biological Sciences, University of Wollongong, Northfields Avenue, 2522, Wollongong, NSW, Australia aut NATIONALLICENCE 700 1- Roberts Kate Illawarra Health and Medical Research Institute, School of Biological Sciences, University of Wollongong, Northfields Avenue, 2522, Wollongong, NSW, Australia aut NATIONALLICENCE 700 1- Lee Jodi Illawarra Health and Medical Research Institute, School of Biological Sciences, University of Wollongong, Northfields Avenue, 2522, Wollongong, NSW, Australia aut NATIONALLICENCE 700 1- Ecroyd Heath Illawarra Health and Medical Research Institute, School of Biological Sciences, University of Wollongong, Northfields Avenue, 2522, Wollongong, NSW, Australia aut NATIONALLICENCE 773 0- Cell Stress and Chaperones Springer Netherlands 18/2(2013-03-01), 251-257 1355-8145 18:2<251 2013 18 12192 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer