Progression and Differentiation of Alveolar Rhabdomyosarcoma Is Regulated by PAX7 Transcription Factor—Significance of Tumor Subclones
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, w...
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2021-07-01
|
| Series: | Cells |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2073-4409/10/8/1870 |
| _version_ | 1827685672397307904 |
|---|---|
| author | Klaudia Skrzypek Grażyna Adamek Marta Kot Bogna Badyra Marcin Majka |
| author_facet | Klaudia Skrzypek Grażyna Adamek Marta Kot Bogna Badyra Marcin Majka |
| author_sort | Klaudia Skrzypek |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-10T08:55:53Z |
| format | Article |
| id | doaj.art-c8de5b2190414e72b99422e1a1376e5a |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-03-10T08:55:53Z |
| publishDate | 2021-07-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cells |
| spelling | doaj.art-c8de5b2190414e72b99422e1a1376e5a2023-11-22T07:08:29ZengMDPI AGCells2073-44092021-07-01108187010.3390/cells10081870Progression and Differentiation of Alveolar Rhabdomyosarcoma Is Regulated by PAX7 Transcription Factor—Significance of Tumor SubclonesKlaudia Skrzypek0Grażyna Adamek1Marta Kot2Bogna Badyra3Marcin Majka4Department of Transplantation, Faculty of Medicine, Institute of Pediatrics, Jagiellonian University Medical College, 30-663 Krakow, PolandDepartment of Transplantation, Faculty of Medicine, Institute of Pediatrics, Jagiellonian University Medical College, 30-663 Krakow, PolandDepartment of Transplantation, Faculty of Medicine, Institute of Pediatrics, Jagiellonian University Medical College, 30-663 Krakow, PolandDepartment of Transplantation, Faculty of Medicine, Institute of Pediatrics, Jagiellonian University Medical College, 30-663 Krakow, PolandDepartment of Transplantation, Faculty of Medicine, Institute of Pediatrics, Jagiellonian University Medical College, 30-663 Krakow, PolandRhabdomyosarcoma (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.https://www.mdpi.com/2073-4409/10/8/1870PAX7transcription factorsmicroRNAsurface markersdifferentiationtumor progression |
| spellingShingle | Klaudia Skrzypek Grażyna Adamek Marta Kot Bogna Badyra Marcin Majka Progression and Differentiation of Alveolar Rhabdomyosarcoma Is Regulated by PAX7 Transcription Factor—Significance of Tumor Subclones Cells PAX7 transcription factors microRNA surface markers differentiation tumor progression |
| title | Progression and Differentiation of Alveolar Rhabdomyosarcoma Is Regulated by PAX7 Transcription Factor—Significance of Tumor Subclones |
| title_full | Progression and Differentiation of Alveolar Rhabdomyosarcoma Is Regulated by PAX7 Transcription Factor—Significance of Tumor Subclones |
| title_fullStr | Progression and Differentiation of Alveolar Rhabdomyosarcoma Is Regulated by PAX7 Transcription Factor—Significance of Tumor Subclones |
| title_full_unstemmed | Progression and Differentiation of Alveolar Rhabdomyosarcoma Is Regulated by PAX7 Transcription Factor—Significance of Tumor Subclones |
| title_short | Progression and Differentiation of Alveolar Rhabdomyosarcoma Is Regulated by PAX7 Transcription Factor—Significance of Tumor Subclones |
| title_sort | progression and differentiation of alveolar rhabdomyosarcoma is regulated by pax7 transcription factor significance of tumor subclones |
| topic | PAX7 transcription factors microRNA surface markers differentiation tumor progression |
| url | https://www.mdpi.com/2073-4409/10/8/1870 |
| work_keys_str_mv | AT klaudiaskrzypek progressionanddifferentiationofalveolarrhabdomyosarcomaisregulatedbypax7transcriptionfactorsignificanceoftumorsubclones AT grazynaadamek progressionanddifferentiationofalveolarrhabdomyosarcomaisregulatedbypax7transcriptionfactorsignificanceoftumorsubclones AT martakot progressionanddifferentiationofalveolarrhabdomyosarcomaisregulatedbypax7transcriptionfactorsignificanceoftumorsubclones AT bognabadyra progressionanddifferentiationofalveolarrhabdomyosarcomaisregulatedbypax7transcriptionfactorsignificanceoftumorsubclones AT marcinmajka progressionanddifferentiationofalveolarrhabdomyosarcomaisregulatedbypax7transcriptionfactorsignificanceoftumorsubclones |