GENETIC LOCALIZATION OF A NEWLY RECOGNIZED AUTOSOMAL-DOMINANT LIMB-GIRDLE MUSCULAR-DYSTROPHY WITH CARDIAC INVOLVEMENT (LGMD1B) TO CHROMOSOME 1Q11-21

Limb-girdle muscular dystrophy (LGMD) constitutes a clinically and gen etically heterogeneous group of myogenic disorders with a limb-girdle distribution of weakness. One autosomal dominant family, LGMD1A, has b een linked to chromosome 5q, whereas in other autosomal dominant famil ies linkage to this chromosome has been excluded. We studied 58 member s of three families with a newly recognized autosomal dominantly inher ited LGMD with cardiac involvement. A search with highly polymorphic m icrosatellite markers was carried out. The gene for this newly recogni zed dominant form of LGMD was located on chromosome 1q11-21, with a co mbined maximum two-point LOD score >12 at theta = 0.