ca-circSCN8A Promotes HPASMCs Ferroptosis via LLPS Initiated R-Loop

Hypertension. 2025 Jul;82(7):e114-e128. doi: 10.1161/HYPERTENSIONAHA.125.25036. Epub 2025 May 19.

Abstract

Background: Ferroptosis has been implicated in pulmonary hypertension (PH), and chromatin-associated RNAs are increasingly recognized as key regulators of this process. However, the detailed mechanism remains unexplored.

Methods: Bioinformatics, Sanger sequencing, and RNase R digestion were used to identify the upregulation of ca-circSCN8A. Functional gain and loss assays were used to unveil the role of ca-circSCN8A in hypoxic redox-dependent ferroptosis in human pulmonary arterial smooth muscle cells and a PH mice model. Interaction between ca-circSCN8A and FUS was detected via RNA immunoprecipitation and pull-down assays. Fluorescence recovery after photobleaching, ChIRP-qPCR (Chromatin Isolation by RNA Purification followed by Quatitative PCR), malondialdehyde, reduced glutathione, and glutathione were conducted to explore the potential molecular mechanism.

Results: ca-circSCN8A was identified and confirmed to be upregulated in PH. Its overexpression promoted hypoxia-induced ferroptosis in human pulmonary arterial smooth muscle cells. Under hypoxic conditions, ca-circSCN8A recruited EP300 to facilitate the lactylation of FUS (Fused in Sarcoma), triggering the formation of a ca-circSCN8A/FUS/EP300 complex via liquid-liquid phase separation. Liquid-liquid phase separation maintained the stability of the R-loop formed by ca-circSCN8A and ferroptosis-related gene SLC7A11 (solute carrier family 7 member 11) promoter that inhibits its transcription, further result in the disruption of the redox homeostasis and causing ferroptosis in human pulmonary arterial smooth muscle cells.

Conclusions: ca-circSCN8A recruits EP300 to promote the lactylation of FUS, thereby driving liquid-liquid phase separation-mediated complex formation with FUS and EP300. This process enables ca-circSCN8A to form an R-loop with the nonhost SLC7A11 promoter, contributing to the regulation of hypoxia-induced ferroptosis in human pulmonary arterial smooth muscle cells. This study provides the first evidence that circRNAs can form R-loops with nonhost genes in a liquid-liquid phase separation-dependent manner. Our findings highlight ca-circSCN8A as a crucial regulator of ferroptosis in hypoxic PH and a potential therapeutic target for PH.

Keywords: R-loop structures; RNA-binding protein FUS; ferroptosis; hypertension, pulmonary; phase separation.

MeSH terms

  • Animals
  • Disease Models, Animal
  • Ferroptosis* / genetics
  • Ferroptosis* / physiology
  • Humans
  • Hypertension, Pulmonary* / genetics
  • Hypertension, Pulmonary* / metabolism
  • Hypertension, Pulmonary* / pathology
  • Male
  • Mice
  • Muscle, Smooth, Vascular* / metabolism
  • Myocytes, Smooth Muscle* / metabolism
  • Pulmonary Artery* / metabolism
  • Pulmonary Artery* / pathology
  • RNA, Circular* / genetics
  • RNA, Circular* / metabolism

Substances

  • RNA, Circular