WCN23-0154 Cross-talk of Cilia Sensors and mTOR Signaling Pathway in Cystogenesis of Mouse Polycystic Kidney Disease Model

LTN TRUC, S Matsuda, T Miyazaki… - Kidney International …, 2023 - kireports.org
Methods To better understand the cross-talk betweenmTORand PC, we generated an in-
house PKD mouse model, Cd79a-Cre; Tsc1ff (Cd79a-Tsc1 KO hereafter), recapitulating …

Mechanism of cystogenesis by Cd79a-driven, conditional mTOR activation in developing mouse nephrons

L Tran Nguyen Truc, S Matsuda, A Takenouchi… - Scientific Reports, 2023 - nature.com
Polycystic kidney disease (PKD) is a common genetic disorder arising from developmental
and postnatal processes. Defects in primary cilia and their signaling (eg, mTOR) underlie the …

Mechanisms of Cystogenesis by Cd79a-Driven, Conditional mTOR Activation in Mouse Developing Nephron: FR-PO289

LN Tran, S Matsuda, HQT Thuy… - Journal of the …, 2022 - journals.lww.com
Background: Polycystic kidney diseases (PKD) are a common genetic disorder arising from
developmental and/or postnatal processes. Defects of primary cilia and/or their signaling …

Tulp3 regulates renal cystogenesis by trafficking of cystoproteins to cilia

SH Hwang, BN Somatilaka, H Badgandi, VR Palicharla… - Current Biology, 2019 - cell.com
Polycystic kidney disease proteins, polycystin-1 and polycystin-2, localize to primary cilia.
Polycystin knockouts have severe cystogenesis compared to ciliary disruption, whereas …

Cilia-localized counterregulatory signals as drivers of renal cystogenesis

RV Walker, A Maranto, VR Palicharla… - Frontiers in Molecular …, 2022 - frontiersin.org
Primary cilia play counterregulatory roles in cystogenesis—they inhibit cyst formation in the
normal renal tubule but promote cyst growth when the function of polycystins is impaired …

Novel mTORC1 and 2 Signaling Pathways in Polycystic Kidney Disease (PKD)

CL Edelstein… - 2021 - apps.dtic.mil
This proposal will study novel mTORC1 and 2 signaling pathways that mediate ADPKD and
investigate the effects of mTORC1 Raptor knockout, mTORC2 Rictor knockout or combined …

[HTML][HTML] Cyclin-dependent kinase 4 drives cystic kidney disease in the absence of mTORC1 signaling activity

F Grahammer, B Dumoulin, RE Gulieva, H Wu, Y Xu… - Kidney International, 2024 - Elsevier
Progression of cystic kidney disease has been linked to activation of the mTORC1 signaling
pathway. Yet the utility of mTORC1 inhibitors to treat patients with polycystic kidney disease …

Ciliary ARL13B Is a Major Driver of Kidney Cystogenesis: TH-OR35

RE Van Sciver, E Gigante… - Journal of the American …, 2022 - journals.lww.com
Background: Polycystic kidney disease (PKD) is intricately linked to the primary cilium. PKD1
and PKD2 encode for polycystin proteins which localize to cilia, and mutations in these …

Zebrafish Tsc1 reveals functional interactions between the cilium and the TOR pathway

LM DiBella, A Park, Z Sun - Human molecular genetics, 2009 - academic.oup.com
The cell surface organelle called the cilium is essential for preventing kidney cyst formation
and for establishing left–right asymmetry of the vertebrate body plan. Recent advances …

Polycystin-2 (TRPP2) Regulates primary cilium length in LLC-PK1 renal epithelial cells

PL Perez, N Scarinci, HF Cantiello… - BiorXiv, 2020 - biorxiv.org
Abstract Polycystin-2 (PC2, TRPP2) is a Ca2+ permeable non-selective cation channel
whose dysfunction generates autosomal dominant polycystic kidney disease (ADPKD). PC2 …