Hyperactivity of Mek in TNS1 knockouts leads to potential treatments for cystic kidney diseases

ZY Wu, CL Chiu, E Lo, YRJ Lee, S Yamada… - Cell death & disease, 2019 - nature.com
Cystic kidney disease is the progressive development of multiple fluid-filled cysts that may
severely compromise kidney functions and lead to renal failure. TNS1 (tensin-1) knockout …

[HTML][HTML] C-terminal tensin-like (CTEN) knockin alleviates cystic kidney defects in Tensin-1 knockout mice

CL Chiu, SY Hong, Y Tan, YRJ Lee, YP Shih… - Genes & …, 2023 - ncbi.nlm.nih.gov
Tensin-1 (TNS1) is a 220 kD focal adhesion protein that binds to actin filaments, integrin
receptors, small GTPases, tyrosinephosphorylated proteins, and lipids. 1 These binding …

[HTML][HTML] Netrin-1 Overexpression Induces Polycystic Kidney Disease: A Novel Mechanism Contributing to Cystogenesis in Autosomal Dominant Polycystic Kidney …

R Mohamed, Y Liu, AD Kistler, PC Harris… - The American Journal of …, 2022 - Elsevier
Despite recent advances in understanding the pathogenesis of polycystic kidney disease
(PKD), the underlying molecular mechanisms involved in cystogenesis are not fully …

Loss of Tsc1, but not Pten, in renal tubular cells causes polycystic kidney disease by activating mTORC1

J Zhou, J Brugarolas, LF Parada - Human molecular genetics, 2009 - academic.oup.com
Tuberous sclerosis complex (TSC) is a genetic disorder linked to mutations of either the
TSC1 or TSC2 gene, which encode proteins that form a complex to negatively regulate …

Tsc2 mutation induces renal tubular cell nonautonomous disease

P Kumar, F Zadjali, Y Yao, D Johnson, B Siroky… - Genes & …, 2022 - Elsevier
TSC renal cystic disease is poorly understood and has no approved treatment. In a new
principal cell-targeted murine model of Tsc cystic disease, the renal cystic epithelium is …

The MEK inhibitor trametinib ameliorates kidney fibrosis by suppressing ERK1/2 and mTORC1 signaling

P Andrikopoulos, J Kieswich, S Pacheco… - Journal of the …, 2019 - journals.lww.com
Background During kidney fibrosis, a hallmark and promoter of CKD (regardless of the
underlying renal disorder leading to CKD), the extracellular-regulated kinase 1/2 (ERK1/2) …

mTOR activation initiates renal cell carcinoma development by coordinating ERK and p38MAPK

H Wu, D He, S Biswas, M Shafiquzzaman, X Zhou… - Cancer Research, 2021 - AACR
Renal cell carcinoma (RCC) mainly originates from renal proximal tubules. Intriguingly,
disruption of genes frequently mutated in human RCC samples thus far has only generated …

Blocking rpS6 phosphorylation exacerbates Tsc1 deletion–induced kidney growth

H Wu, J Chen, J Xu, Z Dong, O Meyuhas… - Journal of the …, 2016 - journals.lww.com
The molecular mechanisms underlying renal growth and renal growth–induced nephron
damage remain poorly understood. Here, we report that in murine models, deletion of the …

Not all kidney cysts are created equal: a distinct renal cystogenic mechanism in tuberous sclerosis complex (TSC)

M Soleimani - Frontiers in Physiology, 2023 - frontiersin.org
Tuberous Sclerosis Complex (TSC) is an autosomal dominant genetic disease caused by
mutations in either TSC1 or TSC2 genes. Approximately, two million individuals suffer from …

Notch transactivates Rheb to maintain the multipotency of TSC-null cells

JH Cho, B Patel, S Bonala, S Manne, Y Zhou… - Nature …, 2017 - nature.com
Differentiation abnormalities are a hallmark of tuberous sclerosis complex (TSC)
manifestations; however, the genesis of these abnormalities remains unclear. Here we …