CONFIRMATION OF LOCUS HETEROGENEITY IN THE PURE FORM OF FAMILIAL SPASTIC PARAPLEGIA

Familial spastic paraplegia (FSP), characterized by progressive spasti city of the lower extremities, is in its ''pure'' form generally of au tosomal dominant inheritance pattern, Hazan et al. [Nat Genet 5:163-16 7, 1993] reported tight linkage of a large FSP family to the highly po lymorphic microsatellite marker D14S269 with z ((0)) = 8.49 at (0) = 0 .00 They further demonstrated evidence for locus heterogeneity when th ey showed that 2 FSP families were unlinked to this region, We have su bsequently studied 4 FSP families (3 American, one British) and exclud ed the disease locus in these families for approximately 30 cM on eith er side of D14S269, thereby confirming evidence for locus heterogeneit y within the spastic paraplegia diagnostic classification. (C) 1995 Wi ley-Liss, Inc.