ARPKD and early manifestations of ADPKD: the original polycystic kidney disease and phenocopies

C Bergmann - Pediatric Nephrology, 2015 - Springer
Renal cysts are clinically and genetically heterogeneous conditions. Polycystic kidney
disease (PKD) is common and its characterization has paved the way for the identification of …

Functional aspects of primary cilia in signaling, cell cycle and tumorigenesis

SG Basten, RH Giles - Cilia, 2013 - Springer
Dysfunctional cilia underlie a broad range of cellular and tissue phenotypes and can
eventually result in the development of ciliopathies: pathologically diverse diseases that …

The hallmarks of cancer: relevance to the pathogenesis of polycystic kidney disease

T Seeger-Nukpezah, DM Geynisman… - Nature Reviews …, 2015 - nature.com
Autosomal dominant polycystic kidney disease (ADPKD) is a progressive inherited disorder
in which renal tissue is gradually replaced with fluid-filled cysts, giving rise to chronic kidney …

Endothelial dysfunction and oxidative stress in polycystic kidney disease

J Klawitter, BY Reed-Gitomer… - American Journal …, 2014 - journals.physiology.org
Cardiovascular disease (CVD) is the leading cause of premature mortality in ADPKD
patients. The aim was to identify potential serum biomarkers associated with the severity of …

Dopamine receptor type 5 in the primary cilia has dual chemo-and mechano-sensory roles

S Abdul-Majeed, SM Nauli - Hypertension, 2011 - Am Heart Assoc
Polycystic kidney disease is characterized by cardiovascular irregularities, including
hypertension. Dopamine, a circulating hormone, is implicated in essential hypertension in …

Mechanisms regulating cilia growth and cilia function in endothelial cells

S Abdul-Majeed, BC Moloney, SM Nauli - Cellular and Molecular Life …, 2012 - Springer
The primary cilium is an important sensory organelle present in most mammalian cells. Our
current studies aim at examining intracellular molecules that regulate cilia length and/or cilia …

Renal-retinal ciliopathy gene Sdccag8 regulates DNA damage response signaling

R Airik, GG Slaats, Z Guo, AC Weiss… - Journal of the …, 2014 - journals.lww.com
Nephronophthisis-related ciliopathies (NPHP-RCs) are developmental and degenerative
kidney diseases that are frequently associated with extrarenal pathologies such as retinal …

Epithelial proliferation and cell cycle dysregulation in kidney injury and disease

K Lee, GL Gusella, JC He - Kidney international, 2021 - Elsevier
Various cellular insults and injury to renal epithelial cells stimulate repair mechanisms to
adapt and restore the organ homeostasis. Renal tubular epithelial cells are endowed with …

Educational paper: ciliopathies

C Bergmann - European journal of pediatrics, 2012 - Springer
Cilia are antenna-like organelles found on the surface of most cells. They transduce
molecular signals and facilitate interactions between cells and their environment. Ciliary …

The role of the cilium in normal and abnormal cell cycles: emphasis on renal cystic pathologies

J Pan, T Seeger-Nukpezah, EA Golemis - Cellular and Molecular Life …, 2013 - Springer
The primary cilium protrudes from the cell surface and acts as a sensor for chemical and
mechanical growth cues, with receptors for a number of growth factors (PDGFα, Hedgehog …