Literature DB >> 29364281

A Simple and Low-cost Assay for Measuring Ambulation in Mouse Models of Muscular Dystrophy.

Elizabeth M Gibbs1, Rachelle H Crosbie-Watson2.   

Abstract

Measuring functional outcomes in the treatment of muscular dystrophy is an essential aspect of preclinical testing. The assessment of voluntary ambulation in mouse models is a non-invasive and reproducible activity assay that is directly analogous to measures of patient ambulation such as the 6-minute walk test and related mobility scores. Many common methods for testing mouse ambulation speed and distance are based on the open field test, where an animal's free movement within an arena is measured over time. One major downside to this approach is that commercial software and equipment for high-resolution motion tracking is expensive and may require transferring mice to specialized facilities for testing. Here, we describe a low-cost, video-based system for measuring mouse ambulation that utilizes free and open-source software. Using this protocol, we demonstrate that voluntary ambulation in the dystrophin-null mdx mouse model for Duchenne muscular dystrophy (DMD) is decreased relative to wild-type mouse activity. In mdx mice expressing the utrophin transgene, these activity deficits are not observed and the total distance traveled is indistinguishable from wild-type mice. This method is effective for measuring changes in voluntary ambulation associated with dystrophic pathology, and provides a versatile platform that can be readily adapted to diverse research settings.

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Year:  2017        PMID: 29364281      PMCID: PMC5908392          DOI: 10.3791/56772

Source DB:  PubMed          Journal:  J Vis Exp        ISSN: 1940-087X            Impact factor:   1.355


  13 in total

Review 1.  A hitchhiker's guide to behavioral analysis in laboratory rodents.

Authors:  N Sousa; O F X Almeida; C T Wotjak
Journal:  Genes Brain Behav       Date:  2006       Impact factor: 3.449

2.  Intrasession and intersession habituation in mice: from inbred strain variability to linkage analysis.

Authors:  Valerie J Bolivar
Journal:  Neurobiol Learn Mem       Date:  2009-09       Impact factor: 2.877

3.  Use of the six-minute walk test to characterize golden retriever muscular dystrophy.

Authors:  Austin R Acosta; Emiko Van Wie; William B Stoughton; Amanda K Bettis; Heather H Barnett; Nicholas R LaBrie; Cynthia J Balog-Alvarez; Peter P Nghiem; Kevin J Cummings; Joe N Kornegay
Journal:  Neuromuscul Disord       Date:  2016-10-03       Impact factor: 4.296

4.  Suite of clinically relevant functional assays to address therapeutic efficacy and disease mechanism in the dystrophic mdx mouse.

Authors:  Yafeng Song; Shira T Rosenblum; Leon Morales; Mihail Petrov; Christopher Greer; Samantha Globerman; Hansell H Stedman
Journal:  J Appl Physiol (1985)       Date:  2016-12-08

5.  North Star Ambulatory Assessment, 6-minute walk test and timed items in ambulant boys with Duchenne muscular dystrophy.

Authors:  Elena Mazzone; Diego Martinelli; Angela Berardinelli; Sonia Messina; Adele D'Amico; Gessica Vasco; Marion Main; Luca Doglio; Luisa Politano; Filippo Cavallaro; Silvia Frosini; Luca Bello; Adelina Carlesi; Anna Maria Bonetti; Elisabetta Zucchini; Roberto De Sanctis; Marianna Scutifero; Flaviana Bianco; Francesca Rossi; Maria Chiara Motta; Annalisa Sacco; Maria Alice Donati; Tiziana Mongini; Antonella Pini; Roberta Battini; Elena Pegoraro; Marika Pane; Elisabetta Pasquini; Claudio Bruno; Giuseppe Vita; Chiara de Waure; Enrico Bertini; Eugenio Mercuri
Journal:  Neuromuscul Disord       Date:  2010-07-14       Impact factor: 4.296

6.  Multivariate evaluation of the functional recovery obtained by the overexpression of utrophin in skeletal muscles of the mdx mouse.

Authors:  Jean-Marie Gillis
Journal:  Neuromuscul Disord       Date:  2002-10       Impact factor: 4.296

7.  Microtubule binding distinguishes dystrophin from utrophin.

Authors:  Joseph J Belanto; Tara L Mader; Michael D Eckhoff; Dana M Strandjord; Glen B Banks; Melissa K Gardner; Dawn A Lowe; James M Ervasti
Journal:  Proc Natl Acad Sci U S A       Date:  2014-03-31       Impact factor: 11.205

8.  High levels of sarcospan are well tolerated and act as a sarcolemmal stabilizer to address skeletal muscle and pulmonary dysfunction in DMD.

Authors:  Elizabeth M Gibbs; Jamie L Marshall; Eva Ma; Thien M Nguyen; Grace Hong; Jessica S Lam; Melissa J Spencer; Rachelle H Crosbie-Watson
Journal:  Hum Mol Genet       Date:  2016-12-15       Impact factor: 6.150

9.  Sarcolemma-localized nNOS is required to maintain activity after mild exercise.

Authors:  Yvonne M Kobayashi; Erik P Rader; Robert W Crawford; Nikhil K Iyengar; Daniel R Thedens; John A Faulkner; Swapnesh V Parikh; Robert M Weiss; Jeffrey S Chamberlain; Steven A Moore; Kevin P Campbell
Journal:  Nature       Date:  2008-10-26       Impact factor: 49.962

10.  Behavioral and locomotor measurements using an open field activity monitoring system for skeletal muscle diseases.

Authors:  Kathleen S Tatem; James L Quinn; Aditi Phadke; Qing Yu; Heather Gordish-Dressman; Kanneboyina Nagaraju
Journal:  J Vis Exp       Date:  2014-09-29       Impact factor: 1.355

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1.  Sulfadiazine Plus Pyrimethamine Therapy Reversed Multiple Behavioral and Neurocognitive Changes in Long-Term Chronic Toxoplasmosis by Reducing Brain Cyst Load and Inflammation-Related Alterations.

Authors:  Barrios Leda Castaño; Andrea Alice Silva; Lina L Hernandez-Velasco; Ana Paula Da Silva Pinheiro; Daniel Gibaldi; José Roberto Mineo; Neide Maria Silva; Joseli Lannes-Vieira
Journal:  Front Immunol       Date:  2022-04-27       Impact factor: 8.786

2.  Loss of sarcospan exacerbates pathology in mdx mice, but does not affect utrophin amelioration of disease.

Authors:  Elizabeth M Gibbs; Jackie L McCourt; Kara M Shin; Katherine G Hammond; Jamie L Marshall; Rachelle H Crosbie
Journal:  Hum Mol Genet       Date:  2021-04-26       Impact factor: 6.150

3.  Rodent Activity Detector (RAD), an Open Source Device for Measuring Activity in Rodent Home Cages.

Authors:  Bridget A Matikainen-Ankney; Marcial Garmendia-Cedillos; Mohamed Ali; Jonathan Krynitsky; Ghadi Salem; Nanami L Miyazaki; Tom Pohida; Alexxai V Kravitz
Journal:  eNeuro       Date:  2019-07-10
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