Decipherin molecular cues from nephronopthisis using kidney organoids derived from iPSC

Kidney organoids lacking NPHP1 demonstrate that gene loss triggers a cascade of deleterious processes, encompassing disrupted primary ciliary signaling, defective autophagy, mitochondrial impairment, and metabolic perturbations.

However, the temporal sequence of these events remains unresolved, as does the mechanistic pathway through which these cellular dysfunctions converge to increase inflammatory responses and fibrotic remodeling in NPHP -/- kidney organoids when compared to controls.