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Interplay between DYRK1A dosage and aneuploidy-induced neuropathology in Down syndrome - PubMed

8 hours ago
  • #Down syndrome neuropathology
  • #NLRP3 inflammasome activation
  • #DYRK1A gene dosage
  • Study investigates neuron reduction and apoptosis in Down syndrome using patient-derived iPSCs.
  • Trisomies 13, 18, and 21 cause protein aggregate accumulation in neurons due to stress.
  • DYRK1A overdosage in trisomy 21 astrocytes triggers NLRP3 inflammasome, releasing inflammatory cytokines.
  • Combination of protein aggregates and inflammation enhances neuronal apoptosis in trisomy 21.
  • Correcting DYRK1A copy number with chemical chaperone treatment rescues apoptotic neural death.