Centrosome amplification disrupts renal development and causes cystogenesis

LK Dionne, K Shim, M Hoshi, T Cheng, J Wang… - Journal of Cell …, 2018 - rupress.org
LK Dionne, K Shim, M Hoshi, T Cheng, J Wang, V Marthiens, A Knoten, R Basto, S Jain
Journal of Cell Biology, 2018rupress.org
Centrosome number is tightly controlled to ensure proper ciliogenesis, mitotic spindle
assembly, and cellular homeostasis. Centrosome amplification (the formation of excess
centrosomes) has been noted in renal cells of patients and animal models of various types
of cystic kidney disease. Whether this defect plays a causal role in cystogenesis remains
unknown. Here, we investigate the consequences of centrosome amplification during kidney
development, homeostasis, and after injury. Increasing centrosome number in vivo …
Centrosome number is tightly controlled to ensure proper ciliogenesis, mitotic spindle assembly, and cellular homeostasis. Centrosome amplification (the formation of excess centrosomes) has been noted in renal cells of patients and animal models of various types of cystic kidney disease. Whether this defect plays a causal role in cystogenesis remains unknown. Here, we investigate the consequences of centrosome amplification during kidney development, homeostasis, and after injury. Increasing centrosome number in vivo perturbed proliferation and differentiation of renal progenitors, resulting in defective branching morphogenesis and renal hypoplasia. Centrosome amplification disrupted mitotic spindle morphology, ciliary assembly, and signaling pathways essential for the function of renal progenitors, highlighting the mechanisms underlying the developmental defects. Importantly, centrosome amplification was sufficient to induce rapid cystogenesis shortly after birth. Finally, we discovered that centrosome amplification sensitized kidneys in adult mice, causing cystogenesis after ischemic renal injury. Our study defines a new mechanism underlying the pathogenesis of renal cystogenesis, and identifies a potentially new cellular target for therapy.
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