title: Characterization of a YAC contig containing the NBCCS locus and a novel Kruppel-type zinc finger sequence on chromosome segment 9q22.3.
abstract: Gorlin's syndrome or nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant disorder characterized by a familial or hereditary predisposition to basal cell carcinomas (generally multiple and of early onset), odontogenic keratocysts (jaw cysts), palmar and plantar pits, 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 by cytogenetic evidence of deletions in this region in patients with the syndrome. This is supported by loss of heterozygosity in tumors of polymorphic marker loci flanked by D9S197 and D9S180. We have utilized sequence tagged site (STS) mapping and somatic cell hybrid panel analysis to construct two overlapping yeast artificial chromosome (YAC) contigs spanning this region of the genome. We used the YAC contigs to identify a new zinc finger gene containing a highly informative microsatellite locus.
authors.affiliation: Intramural Research Support Program, SAIC Frederick, National Cancer Institute, Frederick Cancer Research and Development Center, Frederick, Maryland, USA.
authors.forename: A
authors.initials: A
authors.lastname: Chidambaram
authors.forename: M
authors.initials: M
authors.lastname: Gailani
authors.forename: B
authors.initials: B
authors.lastname: Gerrard
authors.forename: C
authors.initials: C
authors.lastname: Stewart
authors.forename: A
authors.initials: A
authors.lastname: Goldstein
authors.forename: I
authors.initials: I
authors.lastname: Chumakov
authors.forename: A E
authors.initials: AE
authors.lastname: Bale
authors.forename: M
authors.initials: M
authors.lastname: Dean
mesh_terms: D000595:Amino Acid Sequence; D001478:Basal Cell Nevus Syndrome / Q000235:genetics*; D001483:Base Sequence; D002874:Chromosome Mapping; D018244:Chromosomes, Artificial, Yeast / Q000235:genetics*; D002899:Chromosomes, Human, Pair 9 / Q000235:genetics*; D003360:Cosmids; D018899:CpG Islands; D018076:DNA, Complementary; D004268:DNA-Binding Proteins / Q000235:genetics*; D017353:Gene Deletion; D015698:Genomic Library; D006579:Heterozygote; D006801:Humans; D051741:Kruppel-Like Transcription Factors; D018895:Microsatellite Repeats; D008969:Molecular Sequence Data; D016133:Polymerase Chain Reaction; D012097:Repressor Proteins; D017422:Sequence Analysis, DNA; D016324:Sequence Tagged Sites; D014157:Transcription Factors / Q000235:genetics*; D016335:Zinc Fingers / Q000235:genetics*
chemical_list: D018076:DNA, Complementary; D004268:DNA-Binding Proteins; D051741:Kruppel-Like Transcription Factors; D012097:Repressor Proteins; D014157:Transcription Factors