RRC ID 49557
著者 Noguchi K, Wakai K, Kiyono T, Kawabe M, Yoshikawa K, Hashimoto-Tamaoki T, Kishimoto H, Nakano Y.
タイトル Molecular analysis of keratocystic odontogenic tumor cell lines derived from sporadic and basal cell nevus syndrome patients.
ジャーナル Int J Oncol
Abstract Keratocystic odontogenic tumor (KCOT) is a benign tumor often associated with basal cell nevus syndrome (BCNS). Mutations in Patched 1 (PTCH1), the Hedgehog (Hh) receptor, are responsible for BCNS. BCNS is distinguished by morphological anomalies and predisposition to benign and malignant tumors, including medulloblastoma, basal cell carcinoma, KCOT and ovarian fibromas. Among these tumors, KCOT is the least well studied because a suitable model system is not available for its investigation. To enable KCOT to be studied, we established two KCOT cell lines, one from a BCNS case (designated as iKCOT1) and one from a sporadic KCOT case (designated as sKCOT1). The BCNS‑derived KCOT cell line, iKCOT1, retained a germline-mutated PTCH1 allele and a wild-type PTCH1 allele. The sporadic KCOT-derived KCOT cell line, sKCOT1, had different loss-of-function PTCH1 mutations on both alleles. Both cell lines expressed stem cell markers (CD44, SOX2 and BMI1), mesenchymal cell markers (CDH2, VIM and SNAI2) and a neurogenic marker (NEFL). Culture of the cell lines in high calcium concentration media induced expression of epithelial cell and keratinocyte marker proteins (CDH1, CLDN1, KRT10 and IVL). Parakeratosis, which is characteristic for KCOTs, was observed in 2-D cultures. The similarities in protein expression patterns between the two cell lines suggested that common mechanisms underlie the development of both types of KCOT and a probable common origin of KCOT cells.
巻・号 51(6)
ページ 1731-1738
公開日 2017-12-1
DOI 10.3892/ijo.2017.4146
PMID 29039452
MeSH Adult Basal Cell Nevus Syndrome / genetics* Basal Cell Nevus Syndrome / pathology* Calcium / pharmacology Cell Line, Tumor Culture Media Female Humans Keratinocytes / pathology* Odontogenic Tumors / genetics* Odontogenic Tumors / pathology* Patched-1 Receptor / genetics Point Mutation Tumor Cells, Cultured
IF 3.899
引用数 2
リソース情報
遺伝子材料 CSII-CMV-RfA (RDB04386)