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

被引:260
作者
Grounds, Miranda D. [1 ]
Radley, Hannah G. [1 ]
Lynch, Gordon S. [2 ]
Nagaraju, Kanneboyina [3 ]
De Luca, Annarnaria [4 ]
机构
[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;
D O I
10.1016/j.nbd.2008.03.008
中图分类号
Q189 [神经科学];
学科分类号
071006 [神经生物学];
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.
引用
收藏
页码:1 / 19
页数:19
相关论文
共 199 条
[1]
A mouse with targeted ablation of the growth hormone-releasing hormone gene: A new model of isolated growth hormone deficiency [J].
Alba, M ;
Salvatori, R .
ENDOCRINOLOGY, 2004, 145 (09) :4134-4143
[2]
How calcium influx through calcium leak channels is responsible for the elevated levels of calcium-dependent proteolysis in dystrophic myotubes [J].
Alderton, JM ;
Steinhardt, RA .
TRENDS IN CARDIOVASCULAR MEDICINE, 2000, 10 (06) :268-272
[3]
Mechanisms of stretch-induced muscle damage in normal and dystrophic muscle: role of ionic changes [J].
Allen, DG ;
Whitehead, NP ;
Yeung, EW .
JOURNAL OF PHYSIOLOGY-LONDON, 2005, 567 (03) :723-735
[4]
Postnatal myosin heavy chain isoform expression in normal mice and mice null for IIb or IId myosin heavy chains [J].
Allen, DL ;
Leinwand, LA .
DEVELOPMENTAL BIOLOGY, 2001, 229 (02) :383-395
[5]
Systemic delivery of morpholino oligonucleotide restores dystrophin expression bodywide and improves dystrophic pathology [J].
Alter, J ;
Lou, F ;
Rabinowitz, A ;
Yin, HF ;
Rosenfeld, J ;
Wilton, SD ;
Partridge, TA ;
Lu, QL .
NATURE MEDICINE, 2006, 12 (02) :175-177
[6]
Albumin targeting of damaged muscle fibres in the mdx mouse can be monitored by MRI [J].
Amthor, H ;
Egelhof, T ;
McKinnell, I ;
Ladd, ME ;
Janssen, I ;
Weber, J ;
Sinn, H ;
Schrenk, HH ;
Forsting, M ;
Voit, T ;
Straub, V .
NEUROMUSCULAR DISORDERS, 2004, 14 (12) :791-796
[7]
Deflazacort increases laminin expression and myogenic repair, and induces early persistent functional gain in mdx mouse muscular dystrophy [J].
Anderson, JE ;
Weber, M ;
Vargas, C .
CELL TRANSPLANTATION, 2000, 9 (04) :551-564
[8]
Targeted disruption of exon 52 in the mouse dystrophin gene induced muscle Degeneration similar to that observed in Duchenne muscular dystrophy [J].
Araki, E ;
Nakamura, K ;
Nakao, K ;
Kameya, S ;
Kobayashi, O ;
Nonaka, I ;
Kobayashi, T ;
Katsuki, M .
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1997, 238 (02) :492-497
[9]
Persistent and improved functional gain in mdx dystrophic mice after treatment with L-arginine and deflazacort [J].
Archer, Jonathan D. ;
Vargas, Cinthya C. ;
Anderson, Judy E. .
FASEB JOURNAL, 2006, 20 (02) :738-+
[10]
AN APPARATUS TO MEASURE INVIVO BIOMECHANICAL BEHAVIOR OF DORSIFLEXORS AND PLANTARFLEXORS OF MOUSE ANKLE [J].
ASHTONMILLER, JA ;
HE, YD ;
KADHIRESAN, VA ;
MCCUBBREY, DA ;
FAULKNER, JA .
JOURNAL OF APPLIED PHYSIOLOGY, 1992, 72 (03) :1205-1211