Establishment of a cell line from a malignant rhabdoid tumor of the liver lacking the function of two tumor suppressor genes, hSNF5/INI1 and p16.
Malignant rhabdoid tumors (MRT) of the liver are rare. A few liver MRT cell lines have been established but none has been characterized in detail. Here we describe a new MRT cell line from the liver, which is designated MP-MRT-AN, and describe it in detail. Immunohistochemical assays detected the expression of vimentin and cytokeratin but they were negative for neurofilament, desmin, alpha-smooth muscle actin, alpha-sarcomeric actin, and smooth muscle myosin heavy chains SM1 and SM2. RT-PCR assays revealed that this cell line did not express smooth muscle myosin heavy chain isoforms or MyoD1. No aberration was identified in 22q by G-banded analysis; however, the hSNF5/INI1 gene, a suppressor gene of MRT that maps to 22q11.2, was homozygously deleted from exons 1 to 5 in this cell line. Furthermore, the expression of another tumor suppressor gene, p16 (CDKN2A), was not detected by RT-PCR. This raises the possibility that the aggressive phenotype of malignant rhabdoid tumors is caused by the loss of two or more tumor suppressor genes.
Kuroda
Hiroshi
H
Department of Pediatrics, Kyoto City Hospital, Kyoto, 1-2 Higashitakada-cho, Mibu, Nakagyo-ku, Kyoto 604-8845, Japan. hkuroda@hosp.city.kyoto.jp
Moritake
Hiroshi
H
Sawada
Kazumi
K
Kuwahara
Yasumichi
Y
Imoto
Issei
I
Inazawa
Johji
J
Sugimoto
Tohru
T
D000818:Animals; D045744:Cell Line, Tumor; D002868:Chromosomal Proteins, Non-Histone; D002871:Chromosome Banding; D002874:Chromosome Mapping; D002892:Chromosomes, Human, Pair 22; D004268:DNA-Binding Proteins / Q000235:genetics*; D005260:Female; D017353:Gene Deletion; D016147:Genes, Tumor Suppressor; D019942:Genes, p16; D006720:Homozygote; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007633:Keratins / Q000378:metabolism; D008113:Liver Neoplasms / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D051379:Mice; D008819:Mice, Nude; D009368:Neoplasm Transplantation; D020133:Reverse Transcriptase Polymerase Chain Reaction; D018335:Rhabdoid Tumor / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D000071796:SMARCB1 Protein; D014157:Transcription Factors / Q000235:genetics*; D014463:Ultrasonography; D014746:Vimentin / Q000378:metabolism
D002868:Chromosomal Proteins, Non-Histone; D004268:DNA-Binding Proteins; D000071796:SMARCB1 Protein; C513266:SMARCB1 protein, human; C513265:Smarcb1 protein, mouse; D014157:Transcription Factors; D014746:Vimentin; D007633:Keratins