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Novel two MRT cell lines established from multiple sites of a synchronous MRT patient

Background/Aim: Malignant rhabdoid tumor (MRT) is a rare, aggressive neoplasm found in young children, caused by inactivation of a single gene, SNF5 (INI1, SMARCB1). MRT cases with multifocal tumors at diagnosis are categorized as synchronous MRT, often with a germline mutation of SNF5. The aim of t...

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Bibliographic Details
Main Authors: Kuwahara, Yasumichi (Author) , Johann, Pascal-David (Author)
Format: Article (Journal)
Language:English
Published: November 202
In: Anticancer research
Year: 2020, Volume: 40, Issue: 11, Pages: 6159-6170
ISSN:1791-7530
DOI:10.21873/anticanres.14636
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.21873/anticanres.14636
Verlag, lizenzpflichtig, Volltext: http://ar.iiarjournals.org/content/40/11/6159
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Author Notes:Yasumichi Kuwahara, Tomoko Iehara, Eisuke Ichise, Yoshiki Katsumi, Kazutaka Ouchi, Kunihiko Tsuchiya, Mitsuru Miyachi, Eiichi Konishi, Hiroyasu Sasajima, Satoaki Nakamura, Shigehisa Fumino, Tatsuro Tajiri, Pascal D. Johann, Michael C. Frühwald, Tatsushi Yoshida, Tsukasa Okuda and Hajime Hosoi
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Summary:Background/Aim: Malignant rhabdoid tumor (MRT) is a rare, aggressive neoplasm found in young children, caused by inactivation of a single gene, SNF5 (INI1, SMARCB1). MRT cases with multifocal tumors at diagnosis are categorized as synchronous MRT, often with a germline mutation of SNF5. The aim of this study was to establish new models useful in clarifying the biological basis of synchronous MRT. Materials and Methods: We established two novel MRT cell lines, designated as KP-MRT-KS and KP-MRT-KSa, derived from different lesions and at a different time from a synchronous multifocal 7-month-old female MRT patient. Results: Both cells showed typical morphology of MRT, with a compound genomic mutation in exons 2 and 5 of the SNF5 gene. The exon 2 mutation was found in the germline. Conclusion: These cell lines could serve as powerful tools for unveiling the molecular mechanism of refractory synchronous MRT.
Item Description:Gesehen am 04.01.2021
Physical Description:Online Resource
ISSN:1791-7530
DOI:10.21873/anticanres.14636

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