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 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. Genes Chromosom. Cancer 18:212–218, 1997. © 1997 Wiley‐Liss, Inc.

[1]  Michael Dean,et al.  Mutations of the Human Homolog of Drosophila patched in the Nevoid Basal Cell Carcinoma Syndrome , 1996, Cell.

[2]  I. Vořechovský,et al.  A Mammalian patched Homolog Is Expressed in Target Tissues of sonic hedgehog and Maps to a Region Associated with Developmental Abnormalities (*) , 1996, The Journal of Biological Chemistry.

[3]  Wei-Jen Tang,et al.  Two Cytoplasmic Domains of Mammalian Adenylyl Cyclase Form a G- and Forskolin-activated Enzyme in Vitro(*) , 1996, The Journal of Biological Chemistry.

[4]  N. Tommerup,et al.  Isolation and fine mapping of 16 novel human zinc finger-encoding cDNAs identify putative candidate genes for developmental and malignant disorders. , 1995, Genomics.

[5]  J. Weissenbach,et al.  Analysis of 133 meioses places the genes for nevoid basal cell carcinoma (Gorlin) syndrome and Fanconi anemia group C in a 2.6-cM interval and contributes to the fine map of 9q22.3. , 1994, Genomics.

[6]  A. Reis,et al.  A YAC contig spanning the nevoid basal cell carcinoma syndrome, Fanconi anaemia group C, and xeroderma pigmentosum group A loci on chromosome 9q. , 1994, Genomics.

[7]  A. Goldstein,et al.  Localization of the gene for the nevoid basal cell carcinoma syndrome. , 1994, American journal of human genetics.

[8]  S. Povey,et al.  REPORT on the Third International Workshop on Chromosome 9 , 1994 .

[9]  R. Kerschmann,et al.  Parental origin of chromosome 9q22.3-q31 lost in basal cell carcinomas from basal cell nevus syndrome patients. , 1994, Human molecular genetics.

[10]  M. E. Ruaro,et al.  Structure, function, and chromosome mapping of the growth-suppressing human homologue of the murine gas1 gene. , 1994, Proceedings of the National Academy of Sciences of the United States of America.

[11]  A. Bird,et al.  Number of CpG islands and genes in human and mouse. , 1993, Proceedings of the National Academy of Sciences of the United States of America.

[12]  E. Haan,et al.  Further localization of the gene for nevoid basal cell carcinoma syndrome (NBCCS) in 15 Australasian families: linkage and loss of heterozygosity. , 1993, American journal of human genetics.

[13]  A. Goldstein,et al.  Sun exposure and basal cell carcinomas in the nevoid basal cell carcinoma syndrome. , 1993, Journal of the American Academy of Dermatology.

[14]  N. Carter,et al.  Multiple self–healing squamous epitheliomata (ESS1) mapped to chromosome 9q22–q31 in families with common ancestry , 1993, Nature Genetics.

[15]  M. Greene,et al.  Developmental defects in gorlin syndrome related to a putative tumor suppressor gene on chromosome 9 , 1992, Cell.

[16]  J. Martial,et al.  The evolutionarily conserved Krüppel-associated box domain defines a subfamily of eukaryotic multifingered proteins. , 1991, Proceedings of the National Academy of Sciences of the United States of America.

[17]  P. Goodfellow,et al.  Rapid cloning and characterization of new chromosome 10 DNA markers by Alu element-mediated PCR. , 1990, Genomics.

[18]  D. Ledbetter,et al.  Alu polymerase chain reaction: a method for rapid isolation of human-specific sequences from complex DNA sources. , 1989, Proceedings of the National Academy of Sciences of the United States of America.

[19]  J. Martial,et al.  The human genome contains hundreds of genes coding for finger proteins of the Krüppel type. , 1989, DNA.

[20]  Robert J. Gorlin,et al.  Nevoid Basal‐Cell Carcinoma Syndrome , 1987, Medicine.

[21]  J. Devereux,et al.  A comprehensive set of sequence analysis programs for the VAX , 1984, Nucleic Acids Res..

[22]  G. Chenevix-Trench,et al.  Fine deletion mapping on the long arm of chromosome 9 in sporadic and familial basal cell carcinomas. , 1995, Human molecular genetics.

[23]  G. Chenevix-Trench,et al.  The human growth-arrest-specific gene GAS1 maps outside the candidate region of the gene for nevoid basal cell carcinoma syndrome. , 1995, Cytogenetics and Cell Genetics.