Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophy

This review discusses various issues to consider when developing standard operating procedures for pre-clinical studies in the mdx mouse model of Duchenne muscular dystrophy (DMD). The review describes and evaluates a wide range of techniques used to measure parameters of muscle pathology in mdx mic...

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Main Authors: Miranda D. Grounds, Hannah G. Radley, Gordon S. Lynch, Kanneboyina Nagaraju, Annamaria De Luca
Format: Article
Language:English
Published: Elsevier 2008-07-01
Series:Neurobiology of Disease
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S096999610800065X
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author Miranda D. Grounds
Hannah G. Radley
Gordon S. Lynch
Kanneboyina Nagaraju
Annamaria De Luca
author_facet Miranda D. Grounds
Hannah G. Radley
Gordon S. Lynch
Kanneboyina Nagaraju
Annamaria De Luca
author_sort Miranda D. Grounds
collection DOAJ
description This review discusses various issues to consider when developing standard operating procedures for pre-clinical studies in the mdx mouse model of Duchenne muscular dystrophy (DMD). The review describes and evaluates a wide range of techniques used to measure parameters of muscle pathology in mdx mice and identifies some basic techniques that might comprise standardised approaches for evaluation. While the central aim is to provide a basis for the development of standardised procedures to evaluate efficacy of a drug or a therapeutic strategy, a further aim is to gain insight into pathophysiological mechanisms in order to identify other therapeutic targets. The desired outcome is to enable easier and more rigorous comparison of pre-clinical data from different laboratories around the world, in order to accelerate identification of the best pre-clinical therapies in the mdx mouse that will fast-track translation into effective clinical treatments for DMD.
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spelling doaj.art-cfd182d0925842dd818445cac43fe7c52022-12-21T20:26:36ZengElsevierNeurobiology of Disease1095-953X2008-07-01311119Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophyMiranda D. Grounds0Hannah G. Radley1Gordon S. Lynch2Kanneboyina Nagaraju3Annamaria De Luca4School of Anatomy and Human Biology, the University of Western Australia, Perth, Western Australia, Australia; Corresponding author. Fax: +61 8 6488 1051.School of Anatomy and Human Biology, the University of Western Australia, Perth, Western Australia, AustraliaBasic and Clinical Myology Laboratory, Department of Physiology, the University of Melbourne, Victoria, AustraliaResearch Centre for Genetic Medicine, Murine Drug Testing Facility, Children's National Medical Center, Washington, USASezione di Farmacologia, Dipartimento Farmacobiologico, Facoltà di Farmacia, Università di Bari, Bari, ItalyThis review discusses various issues to consider when developing standard operating procedures for pre-clinical studies in the mdx mouse model of Duchenne muscular dystrophy (DMD). The review describes and evaluates a wide range of techniques used to measure parameters of muscle pathology in mdx mice and identifies some basic techniques that might comprise standardised approaches for evaluation. While the central aim is to provide a basis for the development of standardised procedures to evaluate efficacy of a drug or a therapeutic strategy, a further aim is to gain insight into pathophysiological mechanisms in order to identify other therapeutic targets. The desired outcome is to enable easier and more rigorous comparison of pre-clinical data from different laboratories around the world, in order to accelerate identification of the best pre-clinical therapies in the mdx mouse that will fast-track translation into effective clinical treatments for DMD.http://www.sciencedirect.com/science/article/pii/S096999610800065XMdx mouseMuscular dystrophyStandard operating proceduresBiological variationMuscle functionPre-clinical trials
spellingShingle Miranda D. Grounds
Hannah G. Radley
Gordon S. Lynch
Kanneboyina Nagaraju
Annamaria De Luca
Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophy
Neurobiology of Disease
Mdx mouse
Muscular dystrophy
Standard operating procedures
Biological variation
Muscle function
Pre-clinical trials
title Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophy
title_full Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophy
title_fullStr Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophy
title_full_unstemmed Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophy
title_short Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophy
title_sort towards developing standard operating procedures for pre clinical testing in the mdx mouse model of duchenne muscular dystrophy
topic Mdx mouse
Muscular dystrophy
Standard operating procedures
Biological variation
Muscle function
Pre-clinical trials
url http://www.sciencedirect.com/science/article/pii/S096999610800065X
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