PCR analysis of muscular dystrophy in mdx mice

Animal models for human genetic diseases provide a biological system in which many of the basic features of a disease can be studied and in which approaches for therapeutic intervention can be tested. In many cases it is difficult to obtain human disease tissue samples in sufficient quantity or at particular stages of development to use in identifying the underlying pathological basis of the disease or to study the expression and mutation of the disease gene. The availability of an appropriate animal model of a human genetic disease enables exploration of many of these issues in the laboratory and can provide a rich source of information that can be applied to understanding or treating the corresponding human disease. Duchenne and Becker muscular dystrophies (DMD/BMD) are among the most common human genetic diseases. These allelic, X-linked recessive disorders are prevalent due to a high frequency of new mutation, with one-third of all cases showing no prior family history (Moser, 1984). Several mouse models, referred to as mdx mice, are available for the study of DMD/BMD (Bulfield et al., 1984; Chapman et al., 1989).