TNF-NF-κB-p53 axis restricts in vivo survival of hPSC-derived dopamine neurons

Cell. 2024 Jul 11;187(14):3671-3689.e23. doi: 10.1016/j.cell.2024.05.030. Epub 2024 Jun 11.

Abstract

Ongoing, early-stage clinical trials illustrate the translational potential of human pluripotent stem cell (hPSC)-based cell therapies in Parkinson's disease (PD). However, an unresolved challenge is the extensive cell death following transplantation. Here, we performed a pooled CRISPR-Cas9 screen to enhance postmitotic dopamine neuron survival in vivo. We identified p53-mediated apoptotic cell death as a major contributor to dopamine neuron loss and uncovered a causal link of tumor necrosis factor alpha (TNF-α)-nuclear factor κB (NF-κB) signaling in limiting cell survival. As a translationally relevant strategy to purify postmitotic dopamine neurons, we identified cell surface markers that enable purification without the need for genetic reporters. Combining cell sorting and treatment with adalimumab, a clinically approved TNF-α inhibitor, enabled efficient engraftment of postmitotic dopamine neurons with extensive reinnervation and functional recovery in a preclinical PD mouse model. Thus, transient TNF-α inhibition presents a clinically relevant strategy to enhance survival and enable engraftment of postmitotic hPSC-derived dopamine neurons in PD.

Keywords: Parkinson’s disease; TNF-α inhibition; TP53; apoptosis; cell purification; cell survival; cell therapy; dopamine neurons; genetic screen; transplantation.

MeSH terms

  • Animals
  • Apoptosis
  • CRISPR-Cas Systems
  • Cell Survival* / drug effects
  • Disease Models, Animal
  • Dopaminergic Neurons* / metabolism
  • Humans
  • Mice
  • NF-kappa B* / metabolism
  • Parkinson Disease / metabolism
  • Pluripotent Stem Cells / metabolism
  • Signal Transduction
  • Tumor Necrosis Factor-alpha* / metabolism
  • Tumor Suppressor Protein p53* / metabolism

Substances

  • NF-kappa B
  • Tumor Suppressor Protein p53
  • Tumor Necrosis Factor-alpha