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Emery\u2013Dreifuss muscular dystrophy is caused by mutations in emerin (X\u2010linked) or A\u2010type lamins (autosomal dominant). Mutations in A\u2010type lamins also cause limb\u2010girdle muscular dystrophy type 1B, dilated cardiomyopathy with conduction defect, and Charcot\u2013Marie\u2013Tooth disorder type 2B1. They also cause partial lipodystrophy syndromes. The functions of emerin and A\u2010type lamins and the mechanisms of how mutations in these proteins cause tissue\u2010specific diseases are not well understood. The mutated proteins may cause structural damage to cells but may also affect processes such as gene regulation. This review gives an overview of this topic and describes recent advances in identification of disease\u2010causing mutations, studies of cells and tissues from subjects with these diseases, and animal and cell culture models. 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