title: Establishment of an ASPL-TFE3 renal cell carcinoma cell line (S-TFE).
abstract: Xp11 translocation renal cell carcinoma is a rare disease diagnosed in children and adolescents in the advanced stage with an aggressive clinical course. Various gene fusions including the transcription factor E3 (TFE3) gene located on chromosome X cause the tumor. We established an Xp11 translocation renal cell carcinoma cell line from a renal tumor in a 18-y-old Japanese female and named it "S-TFE." The cell line and its xenograft demonstrated definite gene fusion including TFE3. They showed strong nuclear staining for TFE3 in immunohistochemistry, TFE3 gene rearrangement in dual-color, break-apart FISH analysis and ASPL-TFE3 type 1 fusion transcripts detected by RT-PCR and direct DNA sequencing. Although many renal cell carcinoma cell lines have been established and investigated, only a few cell lines are recognized as Xp11.2 translocation carcinoma. S-TFE will be useful to examine the characteristics and drug susceptibility of Xp11 translocation renal cell carcinoma.
authors.affiliation: Department of Urology, Sapporo Medical University, Sapporo, Japan.
authors.forename: Megumi
authors.initials: M
authors.lastname: Hirobe
authors.forename: Naoya
authors.initials: N
authors.lastname: Masumori
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Tanaka
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kitamura
authors.forename: Taiji
authors.initials: T
authors.lastname: Tsukamoto
mesh_terms: D000293:Adolescent; D000595:Amino Acid Sequence; D000818:Animals; D001483:Base Sequence; D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / Q000235:genetics* / Q000378:metabolism; D063646:Carcinogenesis / Q000473:pathology; D002292:Carcinoma, Renal Cell / Q000235:genetics / Q000473:pathology*; D045744:Cell Line, Tumor; D002467:Cell Nucleus / Q000378:metabolism; D049109:Cell Proliferation; D048430:Cell Shape; D004847:Epithelial Cells / Q000473:pathology / Q000502:physiology; D005260:Female; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins; D059785:Karyotype; D007680:Kidney Neoplasms / Q000235:genetics / Q000473:pathology*; D051379:Mice; D008807:Mice, Inbred BALB C; D008819:Mice, Nude; D008969:Molecular Sequence Data; D009368:Neoplasm Transplantation; D015514:Oncogene Proteins, Fusion / Q000235:genetics* / Q000378:metabolism; D017422:Sequence Analysis, DNA; D014178:Translocation, Genetic
chemical_list: C422743:ASPSCR1 protein, human; D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; D047908:Intracellular Signaling Peptides and Proteins; D015514:Oncogene Proteins, Fusion; C069203:TFE3 protein, human
keywords: FISH; TFE3; Xp11.2; cell line; renal neoplasm; translocation; tumorigenesis