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10.3390/cells10081870
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Article

Progression and Differentiation of Alveolar Rhabdomyosarcoma Is Regulated by PAX7 Transcription Factor—Significance of Tumor Subclones

by
Klaudia Skrzypek
*,†,
Grażyna Adamek
†,
Marta Kot
,
Bogna Badyra
and
Marcin Majka
*
Department of Transplantation, Faculty of Medicine, Institute of Pediatrics, Jagiellonian University Medical College, 30-663 Krakow, Poland
*
Authors to whom correspondence should be addressed.
†
Equally contributed.
Cells 2021, 10(8), 1870; https://doi.org/10.3390/cells10081870
Submission received: 11 June 2021 / Revised: 12 July 2021 / Accepted: 20 July 2021 / Published: 23 July 2021
(This article belongs to the Special Issue Advances in Rhabdomyosarcoma)
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Abstract

Rhabdomyosarcoma (RMS), is the most frequent soft tissue tumor in children that originates from disturbances in differentiation process. Mechanisms leading to the development of RMS are still poorly understood. Therefore, by analysis of two RMS RH30 cell line subclones, one subclone PAX7 negative, while the second one PAX7 positive, and comparison with other RMS cell lines we aimed at identifying new mechanisms crucial for RMS progression. RH30 subclones were characterized by the same STR profile, but different morphology, rate of proliferation, migration activity and chemotactic abilities in vitro, as well as differences in tumor morphology and growth in vivo. Our analysis indicated a different level of expression of adhesion molecules (e.g., from VLA and ICAM families), myogenic microRNAs, such as miR-206 and transcription factors, such as MYOD, MYOG, SIX1, and ID. Silencing of PAX7 transcription factor with siRNA confirmed the crucial role of PAX7 transcription factor in proliferation, differentiation and migration of RMS cells. To conclude, our results suggest that tumor cell lines with the same STR profile can produce subclones that differ in many features and indicate crucial roles of PAX7 and ID proteins in the development of RMS.
Keywords: PAX7; transcription factors; microRNA; surface markers; differentiation; tumor progression; rhabdomyosarcoma; cell subclones; cell lines instability PAX7; transcription factors; microRNA; surface markers; differentiation; tumor progression; rhabdomyosarcoma; cell subclones; cell lines instability
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MDPI and ACS Style

Skrzypek, K.; Adamek, G.; Kot, M.; Badyra, B.; Majka, M. Progression and Differentiation of Alveolar Rhabdomyosarcoma Is Regulated by PAX7 Transcription Factor—Significance of Tumor Subclones. Cells 2021, 10, 1870. https://doi.org/10.3390/cells10081870

AMA Style

Skrzypek K, Adamek G, Kot M, Badyra B, Majka M. Progression and Differentiation of Alveolar Rhabdomyosarcoma Is Regulated by PAX7 Transcription Factor—Significance of Tumor Subclones. Cells. 2021; 10(8):1870. https://doi.org/10.3390/cells10081870

Chicago/Turabian Style

Skrzypek, Klaudia, Grażyna Adamek, Marta Kot, Bogna Badyra, and Marcin Majka. 2021. "Progression and Differentiation of Alveolar Rhabdomyosarcoma Is Regulated by PAX7 Transcription Factor—Significance of Tumor Subclones" Cells 10, no. 8: 1870. https://doi.org/10.3390/cells10081870

APA Style

Skrzypek, K., Adamek, G., Kot, M., Badyra, B., & Majka, M. (2021). Progression and Differentiation of Alveolar Rhabdomyosarcoma Is Regulated by PAX7 Transcription Factor—Significance of Tumor Subclones. Cells, 10(8), 1870. https://doi.org/10.3390/cells10081870

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