Proteus syndrome

Cyberian Codex MONDO:0008318

Disorder

  • Name: Proteus syndrome
  • Category: Mendelian
  • Existing deep-research providers: falcon
  • Existing evidence reference count in YAML: 66

Key Pathophysiology Nodes

  • Somatic AKT1 p.E17K activation in mosaic cell clones
  • Elevated PI3K/AKT signaling in AKT1-mutant cells
  • Increased pAKT signaling in AKT1-mutant lesion cells
  • Non-cell-autonomous pAKT activation in adjacent cells
  • AKT overactivation induces excessive vasculogenesis
  • Mural cell dysfunction destabilizes vascular connections

Citation Inventory (for evidence mapping)