A scalable organoid model of human autosomal dominant polycystic kidney disease for disease mechanism and drug discovery

T Tran, CJ Song, T Nguyen, SY Cheng, JA McMahon… - Cell Stem Cell, 2022 - cell.com
Human pluripotent stem-cell-derived organoids are models for human development and
disease. We report a modified human kidney organoid system that generates thousands of …

Embryonic kidney development, stem cells and the origin of Wilms tumor

H Li, P Hohenstein, S Kuure - Genes, 2021 - mdpi.com
The adult mammalian kidney is a poorly regenerating organ that lacks the stem cells that
could replenish functional homeostasis similarly to, eg, skin or the hematopoietic system …

Small GTPases and their role in vascular disease

A Flentje, R Kalsi, TS Monahan - International journal of molecular …, 2019 - mdpi.com
Over eighty million people in the United States have cardiovascular disease that can affect
the heart causing myocardial infarction; the carotid arteries causing stroke; and the lower …

Actin polymerization controls cilia-mediated signaling

ML Drummond, M Li, E Tarapore, TTL Nguyen… - Journal of Cell …, 2018 - rupress.org
Primary cilia are polarized organelles that allow detection of extracellular signals such as
Hedgehog (Hh). How the cytoskeleton supporting the cilium generates and maintains a …

Defective endothelial cell migration in the absence of Cdc42 leads to capillary-venous malformations

B Laviña, M Castro, C Niaudet, B Cruys… - …, 2018 - journals.biologists.com
Formation and homeostasis of the vascular system requires several coordinated cellular
functions, but their precise interplay during development and their relative importance for …

IRSp53 controls plasma membrane shape and polarized transport at the nascent lumen in epithelial tubules

S Bisi, S Marchesi, A Rizvi, D Carra… - Nature …, 2020 - nature.com
It is unclear whether the establishment of apical–basal cell polarity during the generation of
epithelial lumens requires molecules acting at the plasma membrane/actin interface. Here …

A polarizing issue: diversity in the mechanisms underlying apico-basolateral polarization in vivo

MA Pickett, VF Naturale… - Annual review of cell and …, 2019 - annualreviews.org
Polarization along an apico-basolateral axis is a hallmark of epithelial cells and is essential
for their selective barrier and transporter functions, as well as for their ability to provide …

[HTML][HTML] Exploring key challenges of understanding the pathogenesis of kidney disease in Bardet–Biedl syndrome

E Marchese, M Ruoppolo, A Perna, G Capasso… - Kidney international …, 2020 - Elsevier
Bardet–Biedl syndrome (BBS) is a rare pleiotropic inherited disorder known as a ciliopathy.
Kidney disease is a cardinal clinical feature; however, it is one of the less investigated traits …

Rho GTPases as key molecular players within intestinal mucosa and GI diseases

R Pradhan, PA Ngo, LC Martínez-Sánchez… - Cells, 2021 - mdpi.com
Rho proteins operate as key regulators of the cytoskeleton, cell morphology and trafficking.
Acting as molecular switches, the function of Rho GTPases is determined by guanosine …

Genetics of childhood steroid sensitive nephrotic syndrome: an update

BM Lane, R Cason, CI Esezobor… - Frontiers in …, 2019 - frontiersin.org
Advances in genome science in the last 20 years have led to the discovery of over 50 single
gene causes and genetic risk loci for steroid resistant nephrotic syndrome (SRNS). Despite …