Expansion of the myotonic dystrophy gene in Italian and Spanish patients.

Melchionda, S; Cobo, A; Gennarelli, M; Martorell, L; Fattorini, C; Baiget, M; Lopez de Munain, A; Johnson, K et al. · J Med Genet · 1992

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Abstract

Myotonic dystrophy results from expansion of a (CTG)n repeat at the 3' untranslated region of the myotonin-protein kinase gene. We show here the genomic analysis of 322 symptomatic patients with the cDNA-25 probe detecting disease specific EcoRI restriction fragments. The expansion was found in the majority of Italian and Spanish patients (92%). The implications of these results for the detection of symptomatic patients in southern Europe are discussed.

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