TY - JOUR
T1 - SOD1 oligomers in amyotrophic lateral sclerosis
AU - Choi, Esther S.
AU - Dokholyan, Nikolay V.
N1 - Funding Information:
We thank B.H., C.S., J.R, and J.W. for proofreading the manuscript. We acknowledge support from the National Institutes for Health ( 1R35 GM134864 to N.V.D.) and the Passananti Foundation . The project described was also supported by the National Center for Advancing Translational Sciences, National Institutes of Health , through Grant UL1 TR002014 . The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH.
Publisher Copyright:
© 2020 Elsevier Ltd
PY - 2021/2
Y1 - 2021/2
N2 - Identifying nonnative, trimeric forms of SOD1 trimers as the toxic species, rather than large aggregates revolutionizes our understanding of ALS pathophysiology. Large protein aggregates, what was previously thought as the central cause of neurodegeneration, play protective role and are not responsible for neuronal death. SOD1 trimers are implicated at the molecular, cellular, and organismal level. Understanding the formation of the nonnative trimer and its role in the cell, leading to cell death, holds the key to developing a new standard of therapeutics for ALS and for other neurodegenerative diseases. This review highlights recent advances of knowledge for the role of SOD1 oligomers in ALS.
AB - Identifying nonnative, trimeric forms of SOD1 trimers as the toxic species, rather than large aggregates revolutionizes our understanding of ALS pathophysiology. Large protein aggregates, what was previously thought as the central cause of neurodegeneration, play protective role and are not responsible for neuronal death. SOD1 trimers are implicated at the molecular, cellular, and organismal level. Understanding the formation of the nonnative trimer and its role in the cell, leading to cell death, holds the key to developing a new standard of therapeutics for ALS and for other neurodegenerative diseases. This review highlights recent advances of knowledge for the role of SOD1 oligomers in ALS.
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U2 - 10.1016/j.sbi.2020.12.002
DO - 10.1016/j.sbi.2020.12.002
M3 - Review article
C2 - 33465527
AN - SCOPUS:85099303721
SN - 0959-440X
VL - 66
SP - 225
EP - 230
JO - Current Opinion in Structural Biology
JF - Current Opinion in Structural Biology
ER -