2 CRANIOSYNOSTOTIC SYNDROME LOCI, CROUZON AND JACKSON-WEISS, MAP TO CHROMOSOME-10Q23-Q26

Crouzon syndrome (MIM 123500) is a common autosomal dominant form of c raniosynostosis with shallow orbits, ocular proptosis, and maxillary h ypoplasia. Jackson-Weiss syndrome (MIM 123150) is another autosomal do minant craniosynostosis with highly variable phenotypic expression. Un like Crouzon syndrome, Jackson-Weiss syndrome is associated with foot anomalies. We performed two point linkage and haplotype analyses using 13 dinucleotide repeat markers on chromosome 10, spanning a genetic d istance of 108 cM. The Crouzon syndrome locus (CFD1) maps to the regio n of chromosome 10q2, with the tightest linkage to locus D10S205 (Z = 3.09, ($) over cap theta = 0.00). The Jackson-Weiss syndrome locus in the large Amish pedigree in which the condition was originally describ ed was also linked to the chromosome 10q23-q26 region between loci D10 S190 and D10S186. The D10S209 locus was most strongly linked (Z = 11.2 9, ($) over cap theta = 0.00), (C) 1994 Academic Press, Inc.