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We report two sisters with congenital myotonic dystrophy (CDM) born to a normal mother and an affected father. The congenitally affected daughters had symptoms from birth. The age of onset of DM in the father was 39 years. Analysis of the CTG trinucleotide expansion in this family showed increase in the repeat length with increasing severity, with the smallest expansion in the grandfather and the largest expansion in the younger of the two CDM sisters. This family shows that exceptionally it is possible for CDM to be inherited paternally and refutes the hypothesis that CDM is exclusively of maternal origin. This contradicts several of the previous hypotheses concerning the mechanisms by which the CDM phenotype arises.

Lipases (triacylglycerol acylhydrolase, EC 3.1.1.3) were purified from Aspergillus repens and Euro tium herbariorum NU-2 strain by using a DEAE-Sephadex A-50 column and preparative electrophore sis. The purified enzymes from A. repens and NU-2 had molecular weights estimated by SDS-PAGE to be 38,000 and 65,000, respectively. Lipase from A. repens had a pH optimum of 5.3 and a temperature optimum of 27•Ž, while for the NU-2 strain corresponding values were pH 5.2 and 37•Ž. The specific activity of NU-2 was about twice that of A. repens. Substrate specificity toward olive oil or triolein and positional specificity for hydrolyzing the 1 (3)-position ester bonds of triacylglycerol are discussed for both enzymes.

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