Pamela Vivian Tran

This study shows ADAMTS9 deletion in mice causes polycystic kidney disease by disrupting ciliogenesis and collagen homeostasis, leading to small, cystic kidneys and early lethality in males.

This study shows that CILK1 accumulates in an inactive form during ADPKD progression in human and mouse models.

This study shows that targeting Ogt in ADPKD reduces cyst formation and extends survival by normalizing metabolic pathways and preserving cellular energy production.

The paper shows that SPECC1L regulates F-actin levels, which in turn affects cilia length and palate development, with a genetic interaction with Thm1.

This study shows that Rag GTPases suppress renal cystic disease by inhibiting TFEB, independent of mTORC1, and identifies nuclear TFEB as a better biomarker than mTORC1 activation.

This study shows that Notch3 deletion reduces HIV-1 gene expression and systemic inflammation, improving outcomes in HIV-associated chronic kidney disease.

This study shows that deleting the IFT-A gene Thm1 in ADPKD mouse models has development-specific effects on kidney cysts, inflammation, and cilia length, suggesting environmental factors influence ciliary homeostasis and disease progression.

This study shows that Thm1 deletion in juvenile Pkd2 mice worsens biliary development and PLD, while adult Thm1 deletion has no effect, highlighting distinct roles of IFT-A and IFT-B in liver disease.

This study shows Quinomycin A reduces cyst progression in PKD by inhibiting Notch signaling and normalizing cilia lengths in a mouse model and human cells.

This study identifies Thm2 as a novel locus that interacts with Thm1 and sensitizes to Hedgehog signaling in postnatal skeletal development, leading to osteochondrodysplasia.

This study investigates the roles of Thm1 and Thm2 in cilia function and embryogenesis using mouse embryonic fibroblasts, revealing their distinct and overlapping contributions to ciliary dynamics and signaling.