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Decipherin molecular cues from nephronopthisis using kidney organoids derived from iPSC

GSE306868 Homo sapiens Expression profiling by high throughput sequencing 6 samples Submitted 2026/08/01 Platform GPL24676
Summary
To dissect the pathological mechanisms underpinning juvenile nephronophthisis, we utilized human induced pluripotent stem cells (hiPSCs) to interrogate tubular and ciliary architecture and to delineate the molecular consequences of NPHP1 deficiency. 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.
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Also filed as BioProject PRJNA1312813 and SRA study SRP614740. Searching any of these in the dataset finder brings you back here.

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