Zooming in on Long Non-Coding RNAs in Ewing Sarcoma Pathogenesis
Ewing sarcoma (ES) is a rare aggressive cancer of bone and soft tissue that is mainly characterized by a reciprocal chromosomal translocation. As a result, about 90% of cases express the EWS-FLI1 fusion protein that has been shown to function as an aberrant transcription factor driving sarcomagenesi...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-04-01
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| Series: | Cells |
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| Online Access: | https://www.mdpi.com/2073-4409/11/8/1267 |
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| author | Dave N. T. Aryee Valerie Fock Utkarsh Kapoor Branka Radic-Sarikas Heinrich Kovar |
| author_facet | Dave N. T. Aryee Valerie Fock Utkarsh Kapoor Branka Radic-Sarikas Heinrich Kovar |
| author_sort | Dave N. T. Aryee |
| collection | DOAJ |
| description | Ewing sarcoma (ES) is a rare aggressive cancer of bone and soft tissue that is mainly characterized by a reciprocal chromosomal translocation. As a result, about 90% of cases express the EWS-FLI1 fusion protein that has been shown to function as an aberrant transcription factor driving sarcomagenesis. ES is the second most common malignant bone tumor in children and young adults. Current treatment modalities include dose-intensified chemo- and radiotherapy, as well as surgery. Despite these strategies, patients who present with metastasis or relapse still have dismal prognosis, warranting a better understanding of treatment resistant-disease biology in order to generate better prognostic and therapeutic tools. Since the genomes of ES tumors are relatively quiet and stable, exploring the contributions of epigenetic mechanisms in the initiation and progression of the disease becomes inevitable. The search for novel biomarkers and potential therapeutic targets of cancer metastasis and chemotherapeutic drug resistance is increasingly focusing on long non-coding RNAs (lncRNAs). Recent advances in genome analysis by high throughput sequencing have immensely expanded and advanced our knowledge of lncRNAs. They are non-protein coding RNA species with multiple biological functions that have been shown to be dysregulated in many diseases and are emerging as crucial players in cancer development. Understanding the various roles of lncRNAs in tumorigenesis and metastasis would determine eclectic avenues to establish therapeutic and diagnostic targets. In ES, some lncRNAs have been implicated in cell proliferation, migration and invasion, features that make them suitable as relevant biomarkers and therapeutic targets. In this review, we comprehensively discuss known lncRNAs implicated in ES that could serve as potential biomarkers and therapeutic targets of the disease. Though some current reviews have discussed non-coding RNAs in ES, to our knowledge, this is the first review focusing exclusively on ES-associated lncRNAs. |
| first_indexed | 2024-03-09T11:01:50Z |
| format | Article |
| id | doaj.art-14b4ea011b884485bd36175e3d6b1cba |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-03-09T11:01:50Z |
| publishDate | 2022-04-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cells |
| spelling | doaj.art-14b4ea011b884485bd36175e3d6b1cba2023-12-01T01:12:56ZengMDPI AGCells2073-44092022-04-01118126710.3390/cells11081267Zooming in on Long Non-Coding RNAs in Ewing Sarcoma PathogenesisDave N. T. Aryee0Valerie Fock1Utkarsh Kapoor2Branka Radic-Sarikas3Heinrich Kovar4St. Anna Children’s Cancer Research Institute, 1090 Vienna, AustriaSt. Anna Children’s Cancer Research Institute, 1090 Vienna, AustriaSt. Anna Children’s Cancer Research Institute, 1090 Vienna, AustriaSt. Anna Children’s Cancer Research Institute, 1090 Vienna, AustriaSt. Anna Children’s Cancer Research Institute, 1090 Vienna, AustriaEwing sarcoma (ES) is a rare aggressive cancer of bone and soft tissue that is mainly characterized by a reciprocal chromosomal translocation. As a result, about 90% of cases express the EWS-FLI1 fusion protein that has been shown to function as an aberrant transcription factor driving sarcomagenesis. ES is the second most common malignant bone tumor in children and young adults. Current treatment modalities include dose-intensified chemo- and radiotherapy, as well as surgery. Despite these strategies, patients who present with metastasis or relapse still have dismal prognosis, warranting a better understanding of treatment resistant-disease biology in order to generate better prognostic and therapeutic tools. Since the genomes of ES tumors are relatively quiet and stable, exploring the contributions of epigenetic mechanisms in the initiation and progression of the disease becomes inevitable. The search for novel biomarkers and potential therapeutic targets of cancer metastasis and chemotherapeutic drug resistance is increasingly focusing on long non-coding RNAs (lncRNAs). Recent advances in genome analysis by high throughput sequencing have immensely expanded and advanced our knowledge of lncRNAs. They are non-protein coding RNA species with multiple biological functions that have been shown to be dysregulated in many diseases and are emerging as crucial players in cancer development. Understanding the various roles of lncRNAs in tumorigenesis and metastasis would determine eclectic avenues to establish therapeutic and diagnostic targets. In ES, some lncRNAs have been implicated in cell proliferation, migration and invasion, features that make them suitable as relevant biomarkers and therapeutic targets. In this review, we comprehensively discuss known lncRNAs implicated in ES that could serve as potential biomarkers and therapeutic targets of the disease. Though some current reviews have discussed non-coding RNAs in ES, to our knowledge, this is the first review focusing exclusively on ES-associated lncRNAs.https://www.mdpi.com/2073-4409/11/8/1267Ewing sarcomalong non-coding RNAscompeting endogenous (ce) RNAregulatory RNAbiomarkerstherapeutic targets |
| spellingShingle | Dave N. T. Aryee Valerie Fock Utkarsh Kapoor Branka Radic-Sarikas Heinrich Kovar Zooming in on Long Non-Coding RNAs in Ewing Sarcoma Pathogenesis Cells Ewing sarcoma long non-coding RNAs competing endogenous (ce) RNA regulatory RNA biomarkers therapeutic targets |
| title | Zooming in on Long Non-Coding RNAs in Ewing Sarcoma Pathogenesis |
| title_full | Zooming in on Long Non-Coding RNAs in Ewing Sarcoma Pathogenesis |
| title_fullStr | Zooming in on Long Non-Coding RNAs in Ewing Sarcoma Pathogenesis |
| title_full_unstemmed | Zooming in on Long Non-Coding RNAs in Ewing Sarcoma Pathogenesis |
| title_short | Zooming in on Long Non-Coding RNAs in Ewing Sarcoma Pathogenesis |
| title_sort | zooming in on long non coding rnas in ewing sarcoma pathogenesis |
| topic | Ewing sarcoma long non-coding RNAs competing endogenous (ce) RNA regulatory RNA biomarkers therapeutic targets |
| url | https://www.mdpi.com/2073-4409/11/8/1267 |
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