CuATSM efficacy is independently replicated in a SOD1 mouse model of ALS while unmetallated ATSM therapy fails to reveal benefits

CuATSM efficacy is independently replicated in a SOD1 mouse model of ALS while unmetallated ATSM therapy fails to reveal benefits

A copper chelator known as diacetylbis(N(4)-methylthiosemicarbazonato) copper II (CuATSM), has been reported to be efficacious in multiple transgenic SOD1 models of amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder affecting motor neurons. Here we report that we also observed C...

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Main Authors: Fernando G. Vieira, Theo Hatzipetros, Kenneth Thompson, Andy J. Moreno, Joshua D. Kidd, Valerie R. Tassinari, Beth Levine, Steven Perrin, Alan Gill
Format: Article
Language:English
Published: Elsevier 2017-06-01
Series:IBRO Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S2451830117300080
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spelling doaj-246f87e177af4a04ac08f48d41cbc0512020-11-24T22:08:57ZengElsevierIBRO Reports2451-83012017-06-012C475310.1016/j.ibror.2017.03.001CuATSM efficacy is independently replicated in a SOD1 mouse model of ALS while unmetallated ATSM therapy fails to reveal benefitsFernando G. VieiraTheo HatzipetrosKenneth ThompsonAndy J. MorenoJoshua D. KiddValerie R. TassinariBeth LevineSteven PerrinAlan GillA copper chelator known as diacetylbis(N(4)-methylthiosemicarbazonato) copper II (CuATSM), has been reported to be efficacious in multiple transgenic SOD1 models of amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder affecting motor neurons. Here we report that we also observed CuATSM efficacy on disease onset and progression in a standardized litter-matched and gender-balanced efficacy study using B6SJL-SOD1G93A/1Gur mice. We also report improved survival trends with CuATSM treatment. In addition, we report a lack of efficacy by unmetallated ATSM in the same model using the same standardized study design. These results add to existing evidence supporting an efficacious role for copper delivery using chaperone molecules in mouse models of ALS.http://www.sciencedirect.com/science/article/pii/S2451830117300080
collection DOAJ
language English
format Article
sources DOAJ
author Fernando G. Vieira
Theo Hatzipetros
Kenneth Thompson
Andy J. Moreno
Joshua D. Kidd
Valerie R. Tassinari
Beth Levine
Steven Perrin
Alan Gill
spellingShingle Fernando G. Vieira
Theo Hatzipetros
Kenneth Thompson
Andy J. Moreno
Joshua D. Kidd
Valerie R. Tassinari
Beth Levine
Steven Perrin
Alan Gill
CuATSM efficacy is independently replicated in a SOD1 mouse model of ALS while unmetallated ATSM therapy fails to reveal benefits
IBRO Reports
author_facet Fernando G. Vieira
Theo Hatzipetros
Kenneth Thompson
Andy J. Moreno
Joshua D. Kidd
Valerie R. Tassinari
Beth Levine
Steven Perrin
Alan Gill
author_sort Fernando G. Vieira
title CuATSM efficacy is independently replicated in a SOD1 mouse model of ALS while unmetallated ATSM therapy fails to reveal benefits
title_short CuATSM efficacy is independently replicated in a SOD1 mouse model of ALS while unmetallated ATSM therapy fails to reveal benefits
title_full CuATSM efficacy is independently replicated in a SOD1 mouse model of ALS while unmetallated ATSM therapy fails to reveal benefits
title_fullStr CuATSM efficacy is independently replicated in a SOD1 mouse model of ALS while unmetallated ATSM therapy fails to reveal benefits
title_full_unstemmed CuATSM efficacy is independently replicated in a SOD1 mouse model of ALS while unmetallated ATSM therapy fails to reveal benefits
title_sort cuatsm efficacy is independently replicated in a sod1 mouse model of als while unmetallated atsm therapy fails to reveal benefits
publisher Elsevier
series IBRO Reports
issn 2451-8301
publishDate 2017-06-01
description A copper chelator known as diacetylbis(N(4)-methylthiosemicarbazonato) copper II (CuATSM), has been reported to be efficacious in multiple transgenic SOD1 models of amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder affecting motor neurons. Here we report that we also observed CuATSM efficacy on disease onset and progression in a standardized litter-matched and gender-balanced efficacy study using B6SJL-SOD1G93A/1Gur mice. We also report improved survival trends with CuATSM treatment. In addition, we report a lack of efficacy by unmetallated ATSM in the same model using the same standardized study design. These results add to existing evidence supporting an efficacious role for copper delivery using chaperone molecules in mouse models of ALS.
url http://www.sciencedirect.com/science/article/pii/S2451830117300080
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