Activation of long non-coding RNA NEAT1 leads to survival advantage of multiple myeloma cells by supporting a positive regulatory loop with DNA repair proteins

Haematologica. 2023 Jan 1;108(1):219-233. doi: 10.3324/haematol.2022.281167.

Abstract

Long non-coding RNA NEAT1 is the core structural component of the nuclear paraspeckle (PS) organelles and it has been found to be deregulated in multiple myeloma (MM) patients. Experimental evidence indicated that NEAT1 silencing negatively impacts proliferation and viability of MM cells, both in vitro and in vivo, suggesting a role in DNA damage repair (DDR). In order to elucidate the biological and molecular relevance of NEAT1 upregulation in MM disease we exploited the CRISPR/Cas9 synergistic activation mediator genome editing system to engineer the AMO-1 MM cell line and generate two clones that para-physiologically transactivate NEAT1 at different levels. NEAT1 overexpression is associated with oncogenic and prosurvival advantages in MM cells exposed to nutrient starvation or a hypoxic microenvironment, which are stressful conditions often associated with more aggressive disease phases. Furthermore, we highlighted the NEAT1 involvement in virtually all DDR processes through, at least, two different mechanisms. On one side NEAT1 positively regulates the posttranslational stabilization of essential PS proteins, which are involved in almost all DDR systems, thus increasing their availability within cells. On the other hand, NEAT1 plays a crucial role as a major regulator of a molecular axis that includes ATM and the catalytic subunit of DNA-PK kinase proteins, and their direct targets pRPA32 and pCHK2. Overall, we provided novel important insightsthe role of NEAT1 in supporting MM cells adaptation to stressful conditions by improving the maintenance of DNA integrity. Taken together, our results suggest that NEAT1, and probably PS organelles, could represent a potential therapeutic target for MM treatment.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cell Line, Tumor
  • DNA Repair
  • Humans
  • MicroRNAs* / genetics
  • Multiple Myeloma* / genetics
  • RNA, Long Noncoding* / genetics
  • RNA, Long Noncoding* / metabolism
  • Transcriptional Activation
  • Tumor Microenvironment
  • Up-Regulation

Substances

  • MicroRNAs
  • RNA, Long Noncoding
  • NEAT1 long non-coding RNA, human

Grants and funding

Funding: This work was financially supported by grants from Associazione Italiana Ricerca sul Cancro (AIRC) to AN (IG16722 and IG24365), to RC (IG20614), to RP (IG21585), and to NA (IG24449). NB is funded by the European Research Council under the European Union’s Horizon 2020 research and innovation program (grant agreement no. 817997). This study was partially funded by Italian Ministry of Health-Current research IRCCS 2023 (to AN and to NB). YT acknowledges support by “Piattaforme cellulari per la Ricerca e lo Sviluppo di Terapie Avanzate in Life Science” - Fondo Europeo di Sviluppo Regionale 2014-2020. POR FESR 2014-2020. CB received a 2-year AIRC fellowship "Angela e Luciano Cenino" (id.24140); VKF and DG received a fellowship from the PhD program in Experimental Medicine of Milan University.