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\nMyotonic dystrophy is a neuromuscular disorder characterized by progressive weakness and degeneration of muscle, cardiac and respiratory compromise. The genetic disorder affects a percentage of one in every 8,000 to 11,000 live births, having an overall prevalence of around one in 5,000 to 10,000 people, with a wide range of different mutations being known. Myotonic dystrophy type 1 (DM1) and type 2 (DM2) are caused by abnormal expansion of a trinucleotide repeat in the 3\u2032-untranslated region of a gene on the long arm of chromosome 19, which codes for a protein, dystrophin (See Boulton et al., Trends Genet. 17, 545-547 (2001)).
\nIn DM1, the average number of CCTG trinucleotide repeats in the 3\u2032-untranslated region of the DMPK gene is between 110 and 200 repeats. Patients with DM2 have intermediate expansions (between 40 and 50 repeats), whereas patients with DM1 have the lowest, and the most severe, variation in allele size. The effect of the repeat expansion on the pathogenesis of the disorder has been the subject of intense investigation (See Boulton et al., 2001). The size of the repeat length, which may be expressed in terms of CCTG repeats, is the primary determinant of the clinical phenotype of the disease. Thus, a reduction of the repeat length to below 100 repeats is usually associated with mild myotonia, and no other pathological symptoms.
\nGenotype-phenotype correlation studies have not identified a correlation between the repeat length and clinical phenotype (See Boulton et al., 2001). However, the patient’s age, the number of trinucleotide repeats and the amount of expansion (\u201cload\u201d) of the expanded allele have been suggested to influence the rate of progression.
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