title: The UOK 257 cell line: a novel model for studies of the human Birt-Hogg-Dubé gene pathway.
abstract: The establishment, characterization, and tumorigenicity of a new epithelial cell line (UOK 257) derived from human renal carcinoma of an individual with Birt-Hogg-Dubé (BHD) syndrome are reported. Unlike other established renal tumor cell lines from sporadic renal cell carcinoma, this is the first established renal tumor cell line of BHD, an inheritable neoplastic syndrome. The isolated tumor cells display loss of contact inhibition in vitro, and produce subcutaneous tumors in mouse xenografts. Histopathologic, ultrastructural, and cytogenetic characterizations of the established tumor cells are reported. Cytogenetic analysis using spectral karyotyping on UOK 257 cells revealed 17p loss and a near-triploid and aneuploid karyotype with multiple fluorescence in situ hybridization analysis using a locus-specific gene probe for MYC. The result demonstrates that the established tumor cells consist of two cell populations, one containing four and one containing five copies of the MYC oncogene.
authors.affiliation: Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Building 10, Room 1W-5888, Bethesda, MD 20892, USA.
authors.forename: Youfeng
authors.initials: Y
authors.lastname: Yang
authors.forename: Hesed M
authors.initials: HM
authors.lastname: Padilla-Nash
authors.forename: Manish A
authors.initials: MA
authors.lastname: Vira
authors.forename: Mones S
authors.initials: MS
authors.lastname: Abu-Asab
authors.forename: Daniel
authors.initials: D
authors.lastname: Val
authors.forename: Robert
authors.initials: R
authors.lastname: Worrell
authors.forename: Maria
authors.initials: M
authors.lastname: Tsokos
authors.forename: Maria J
authors.initials: MJ
authors.lastname: Merino
authors.forename: Christian P
authors.initials: CP
authors.lastname: Pavlovich
authors.forename: Thomas
authors.initials: T
authors.lastname: Ried
authors.forename: W Marston
authors.initials: WM
authors.lastname: Linehan
authors.forename: Cathy D
authors.initials: CD
authors.lastname: Vocke
mesh_terms: D002292:Carcinoma, Renal Cell / Q000235:genetics* / Q000473:pathology; D002453:Cell Cycle / Q000502:physiology; D045744:Cell Line, Tumor; D049109:Cell Proliferation; D002869:Chromosome Aberrations; D002886:Chromosomes, Human, Pair 17; D020440:Gene Duplication; D016259:Genes, myc; D006801:Humans; D007621:Karyotyping; D007680:Kidney Neoplasms / Q000235:genetics* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D008954:Models, Biological; D011003:Ploidies; D011518:Proto-Oncogene Proteins / Q000235:genetics* / Q000502:physiology; D017422:Sequence Analysis, DNA; D015398:Signal Transduction / Q000235:genetics; D013577:Syndrome; D025521:Tumor Suppressor Proteins / Q000235:genetics* / Q000502:physiology
chemical_list: C476078:FLCN protein, human; D011518:Proto-Oncogene Proteins; D025521:Tumor Suppressor Proteins