Literature DB >> 28686587

qMotor, a set of rules for sensitive, robust and quantitative measurement of motor performance in mice.

Laura M Luh1, Indrajit Das1, Anne Bertolotti1.   

Abstract

Phenotypic analysis of mouse models of human diseases is essential to understanding the underlying disease mechanisms and to developing therapeutics. Many models of neurodegenerative diseases are associated with motor dysfunction, a powerful readout for the disease. We describe here a set of measures to quantitatively monitor early disease onset and progression. We named this set of rules qMotor because it enables sensitive, robust and quantitative measurement of motor performance in 3 d. qMotor can be used to assess early disease onset, before paralysis, as well as disease progression in diverse mouse models, and can be exploited to define robust and humane experimental end points, thereby reducing animal suffering. As an example, we apply qMotor to SOD1G93A transgenic mice. Early studies with the original transgenic SOD1G93A mice in the hybrid background (B6SJL-Tg(SOD1-G93A) have been criticized because of high noise in this mixed background and because of inadequate study designs. We applied qMotor in SOD1G93A transgenic mice in an inbred C57BL/6J background, hereafter called iSOD1G93A mice, and show a remarkably robust and consistent phenotype in this line that we use to evaluate a therapeutic approach. qMotor is a protocol generically applicable to different mouse models.

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Year:  2016        PMID: 28686587      PMCID: PMC5568708          DOI: 10.1038/nprot.2017.062

Source DB:  PubMed          Journal:  Nat Protoc        ISSN: 1750-2799            Impact factor:   13.491


  15 in total

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Journal:  Nat Med       Date:  2010-09-21       Impact factor: 53.440

4.  Silencing mutant SOD1 using RNAi protects against neurodegeneration and extends survival in an ALS model.

Authors:  G Scott Ralph; Pippa A Radcliffe; Denise M Day; Janine M Carthy; Marie A Leroux; Debbie C P Lee; Liang-Fong Wong; Lynsey G Bilsland; Linda Greensmith; Susan M Kingsman; Kyriacos A Mitrophanous; Nicholas D Mazarakis; Mimoun Azzouz
Journal:  Nat Med       Date:  2005-03-13       Impact factor: 53.440

Review 5.  Lost in translation: treatment trials in the SOD1 mouse and in human ALS.

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Journal:  Neurobiol Dis       Date:  2007-01-03       Impact factor: 5.996

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Authors:  Sean Scott; Janice E Kranz; Jeff Cole; John M Lincecum; Kenneth Thompson; Nancy Kelly; Alan Bostrom; Jill Theodoss; Bashar M Al-Nakhala; Fernando G Vieira; Jeyanthi Ramasubbu; James A Heywood
Journal:  Amyotroph Lateral Scler       Date:  2008

7.  Ablation of the UPR-mediator CHOP restores motor function and reduces demyelination in Charcot-Marie-Tooth 1B mice.

Authors:  Maria Pennuto; Elisa Tinelli; Mariachiara Malaguti; Ubaldo Del Carro; Maurizio D'Antonio; David Ron; Angelo Quattrini; M Laura Feltri; Lawrence Wrabetz
Journal:  Neuron       Date:  2008-02-07       Impact factor: 17.173

8.  Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit.

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9.  Resetting translational homeostasis restores myelination in Charcot-Marie-Tooth disease type 1B mice.

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10.  Optimised and rapid pre-clinical screening in the SOD1(G93A) transgenic mouse model of amyotrophic lateral sclerosis (ALS).

Authors:  Richard J Mead; Ellen J Bennett; Aneurin J Kennerley; Paul Sharp; Claire Sunyach; Paul Kasher; Jason Berwick; Brigitte Pettmann; Guiseppe Battaglia; Mimoun Azzouz; Andrew Grierson; Pamela J Shaw
Journal:  PLoS One       Date:  2011-08-18       Impact factor: 3.240

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Authors:  Zhen Zhong; Laura Grasso; Caroline Sibilla; Tim J Stevens; Nicholas Barry; Anne Bertolotti
Journal:  EMBO J       Date:  2018-03-01       Impact factor: 11.598

2.  Targeting thalamic circuits rescues motor and mood deficits in PD mice.

Authors:  Ying Zhang; Dheeraj S Roy; Yi Zhu; Yefei Chen; Tomomi Aida; Yuanyuan Hou; Chenjie Shen; Nicholas E Lea; Margaret E Schroeder; Keith M Skaggs; Heather A Sullivan; Kyle B Fischer; Edward M Callaway; Ian R Wickersham; Ji Dai; Xiao-Ming Li; Zhonghua Lu; Guoping Feng
Journal:  Nature       Date:  2022-06-08       Impact factor: 69.504

3.  Downregulating carnitine palmitoyl transferase 1 affects disease progression in the SOD1 G93A mouse model of ALS.

Authors:  Michael Sloth Trabjerg; Dennis Christian Andersen; Pam Huntjens; Kirsten Egelund Oklinski; Luise Bolther; Jonas Laugård Hald; Amalie Elton Baisgaard; Kasper Mørk; Nikolaj Warming; Ulla Bismark Kullab; Lona John Kroese; Colin Eliot Jason Pritchard; Ivo Johan Huijbers; John Dirk Vestergaard Nieland
Journal:  Commun Biol       Date:  2021-04-30
  3 in total

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