miRNA-214 to predict progression and survival in ALS

J Neurol Neurosurg Psychiatry. 2025 Jun 12;96(7):716-720. doi: 10.1136/jnnp-2024-335177.

Abstract

Background: Reliable biomarkers are essential for predicting the progression speed and prognosis of patients with amyotrophic lateral sclerosis (ALS). We previously identified NCK-associated protein 1 (NCKAP1) as a critical factor in the defective phagocytosis observed in induced microglia-like cells (iMGs) from patients with rapidly progressive sporadic ALS. This study explored the roles of microRNA (miRNA)-214, which targets the NCKAP1 gene, in the progression of ALS.

Methods: The discovery cohort (n=29) was used to identify miR-214 targeting NCKAP1 genes. The validation cohort (n=132) was used to determine the clinical usability of miR-214 for predicting disease progression speed and survival time.

Results: In the discovery cohort, miR-214 levels were increased in plasma and iMGs from rapidly progressive ALS participants. This finding was validated in another cohort of 132 ALS participants and 30 age-matched healthy volunteers. Plasma miR-214 levels correlated with disease progression, severity and survival, distinguishing between rapidly progressive and slowly progressive ALS. In addition, miR-214 levels also correlated with plasma neurofilament light chain (NfL) and cerebrospinal fluid inflammatory cytokines, showing specific associations with increased NfL and monocyte chemoattractant protein 1 (MCP-1). Survival prediction accuracy improved when miR-214 levels were considered with NfL or MCP-1 levels.

Conclusions: Plasma miRNA-214 could serve as a novel biomarker for predicting the progression and prognosis of ALS.

Keywords: ALS; Personal Satisfaction.

MeSH terms

  • Adaptor Proteins, Signal Transducing / genetics
  • Adult
  • Aged
  • Amyotrophic Lateral Sclerosis* / blood
  • Amyotrophic Lateral Sclerosis* / genetics
  • Amyotrophic Lateral Sclerosis* / mortality
  • Biomarkers / blood
  • Cohort Studies
  • Cytokines / cerebrospinal fluid
  • Disease Progression
  • Female
  • Humans
  • Male
  • MicroRNAs* / blood
  • Microglia / metabolism
  • Middle Aged
  • Neurofilament Proteins / blood
  • Prognosis

Substances

  • MicroRNAs
  • MIRN214 microRNA, human
  • Biomarkers
  • Neurofilament Proteins
  • neurofilament protein L
  • Adaptor Proteins, Signal Transducing
  • Cytokines