CHARACTERIZATION OF A YAC CONTIG CONTAINING THE NBCCS LOCUS AND A NOVEL KRUPPEL-TYPE ZINC-FINGER SEQUENCE ON CHROMOSOME SEGMENT 9Q22.3

Gorlin's syndrome or nevoid basal cell carcinoma syndrome (NBCCS) is a n autosomal dominant disorder characterized by a familial or hereditar y predisposition to basal cell carcinomas (generally multiple and of e arly onset), odontogenic keratocysts (jaw cysts), palmar and plantar p its, a wide variety of developmental defects, as well as cancers such as medulloblastomas and ovarian fibromas. The gene for NBCCS has been mapped to human chromosome region 9q22.1-q31 by linkage analysis and b y cytogenetic evidence of deletions in this region in patients with th e syndrome. This is supported by loss of heterozygosity in tumors of p olymorphic marker loci flanked by D9S197 and D9S180. We have utilized sequence tagged site (STS) mapping and somatic cell hybrid panel analy sis to construct two overlapping yeast artificial chromosome (YAC) con tigs spanning this region of the genome. We used the YAC contigs to id entify a new zinc finger gene containing a highly informative microsat ellite locus. (C) 1997 Wiley-Liss, Inc.