Familial hypertrophic cardiomyopathy (FHC) is a cardiac disorder trans
mitted as an autosomal dominant trait. FHC has been shown to be geneti
cally heterogeneous with less than 50% of published pedigrees being as
sociated with mutations in the beta myosin heavy chain (beta-MHC) gene
on chromosome 14q11-q12. A second locus has recently been reported on
chromosome 1. We examined the segregation of microsatellite markers i
n a French pedigree for which the disease is not linked to beta-MHC ge
ne. We found significant linkage of the disease locus to several (CA)n
repeats located on chromosome 11 (lod scores between +3.3 and +4.98).
The data suggest the localization of the novel FHC gene in a region s
panning 17 centiMorgans.