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Review
. 2022 Aug 11:12:971174.
doi: 10.3389/fonc.2022.971174. eCollection 2022.

Non-coding RNA in rhabdomyosarcoma progression and metastasis

Farah Ramadan  1   2   3   4 , Raya Saab  5   6 , Nader Hussein  4 , Philippe Clézardin  2   3 , Pascale A Cohen  2   3 , Sandra E Ghayad  1   7
Affiliations
Review

Non-coding RNA in rhabdomyosarcoma progression and metastasis

Farah Ramadan et al. Front Oncol. .

Abstract

Rhabdomyosarcoma (RMS) is a soft tissue sarcoma of skeletal muscle differentiation, with a predominant occurrence in children and adolescents. One of the major challenges facing treatment success is the presence of metastatic disease at the time of diagnosis, commonly associated with the more aggressive fusion-positive subtype. Non-coding RNA (ncRNA) can regulate gene transcription and translation, and their dysregulation has been associated with cancer development and progression. MicroRNA (miRNA) are short non-coding nucleic acid sequences involved in the regulation of gene expression that act by targeting messenger RNA (mRNA), and their aberrant expression has been associated with both RMS initiation and progression. Other ncRNA including long non-coding RNA (lncRNA), circular RNA (circRNA) and ribosomal RNA (rRNA) have also been associated with RMS revealing important mechanistic roles in RMS biology, but these studies are still limited and require further investigation. In this review, we discuss the established roles of ncRNA in RMS differentiation, growth and progression, highlighting their potential use in RMS prognosis, as therapeutic agents or as targets of treatment.

Keywords: circRNA; lncRNA; miRNA; non-coding RNAs; prognosis; rRNA; rhabdomyosarcoma; therapeutic targets.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
The different types of ncRNA that are implicated in RMS progression and metastasis. From left to right: lncRNA can be transcribed from different regions of DNA including enhancers and promotors; miRNA are transcribed first as pri-miRNA sequences which are subsequently processed into pre-miRNA and transported into the cytoplasm to complete maturation and bind to target mRNA for silencing or degradation; a pre-mRNA can be back-spliced to form a circRNA; within the nucleolus, rDNA can encode rRNA that regulate protein synthesis within the ribosomal complex. The ncRNA implicated to date in RMS biology and progression are represented within boxes. Created BioRender.com.
Figure 2
Figure 2
miR-1 and miR-206 targets and functions in RMS. Schematic diagram of miR-1 and miR-206 common and unique targets that have been demonstrated in RMS, and their functions and the prognostic value of miR-206 in RMS. Created with BioRender.com.
Figure 3
Figure 3
LncRNA implication in skeletal muscle myogenesis and RMS. Schematic diagram of lncRNA SYISL, Neat1 and Linc-MD1 implicated in skeletal muscle differentiation and proliferation (left panel) and lncRNA MYCNOS-01, MYCNOS-02 and H19 implicated in RMS proliferation biology (right panel). Created with BioRender.com.
Figure 4
Figure 4
CircRNA and rRNA implicated in RMS biology. Schematic representation of circ-ZNF609 and circVAMP3 upregulating RMS cell growth and proliferation, respectively; rRNA from nucleolar organizer regions and pre-RNA correlated with advanced stage and poor prognosis of RMS; as well as ribosomal subunits 45S and 18S promoting RMS proliferation. Created with BioRender.com.
See this image and copyright information in PMC

References

    1. Rudzinski ER, Anderson JR, Hawkins DS, Skapek SX, Parham DM, Teot LA. The world health organization classification of skeletal muscle tumors in pediatric rhabdomyosarcoma a report from the children’s oncology group. Arch Pathol Lab Med (2015) 139:1281–7. doi: 10.5858/arpa.2014-0475-OA - DOI - PMC - PubMed
    1. Parham DM, Barr FG. Classification of rhabdomyosarcoma and its molecular basis. Adv Anat Pathol (2013) 20:387–97. doi: 10.1097/PAP.0b013e3182a92d0d - DOI - PMC - PubMed
    1. Bennicelli JL, Edwards RH, Barr FG. Mechanism for transcriptional gain of function resulting from chromosomal translocation in alveolar rhabdomyosarcoma. Proc Natl Acad Sci U.S.A. (1996) 93:5455–9. doi: 10.1073/pnas.93.11.5455 - DOI - PMC - PubMed
    1. Bennicelli JL, Advani S, Schäfer BW, Barr FG. PAX3 and PAX7 exhibit conserved cis-acting transcription repression domains and utilize a common gain of function mechanism in alveolar rhabdomyosarcoma. Oncogene (1999) 18:4348–56. doi: 10.1038/sj.onc.1202812 - DOI - PubMed
    1. Barr FG. Gene fusions involving PAX and FOX family members in alveolar rhabdomyosarcoma. Oncogene (2001) 20:5736–46. doi: 10.1038/sj.onc.1204599 - DOI - PubMed

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