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

NEUROBIOLOGY OF DISEASE

卷:31

Review

Grounds, Miranda D.¹  Radley, Hannah G.¹  Lynch, Gordon S.²  Nagaraju, Kanneboyina³  De Luca, Annarnaria⁴ 

[1] Univ Western Australia, Sch Anat & Human Biol, Perth, WA 6009, Australia

[2] Univ Melbourne, Dept Physiol, Basic & Clin Myol Lab, Melbourne, Vic 3010, Australia

[3] Childrens Natl Med Ctr, Murine Drug Testing Facil, Med Genet Res Ctr, Washington, DC 20010 USA

[4] Univ Bari, Dipartimento Farmacobiol, Sez Farmacol, Bari, Italy

关键词: mdx mouse;    muscular dystrophy;    standard operating procedures;    biological variation;    muscle function;    pre-clinical trials;   

DOI  :  10.1016/j.nbd.2008.03.008

来源: Elsevier

【 摘 要 】

This review discusses various issues to consider when developing standard operating procedures for preclinical 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. (C) 2008 Elsevier Inc. All rights reserved.

【 授权许可】

Free