The primary cilium as a complex signaling center

NF Berbari, AK O'Connor, CJ Haycraft, BK Yoder - Current biology, 2009 - cell.com
Respect for the primary cilium has undergone a remarkable renaissance over the past
decade, and it is now thought to be an essential regulator of numerous signaling pathways …

Regulation of blood pressure and salt homeostasis by endothelin

DE Kohan, NF Rossi, EW Inscho… - Physiological …, 2011 - journals.physiology.org
Endothelin (ET) peptides and their receptors are intimately involved in the physiological
control of systemic blood pressure and body Na homeostasis, exerting these effects through …

[HTML][HTML] Mapping the NPHP-JBTS-MKS protein network reveals ciliopathy disease genes and pathways

L Sang, JJ Miller, KC Corbit, RH Giles, MJ Brauer… - Cell, 2011 - cell.com
Summary Nephronophthisis (NPHP), Joubert (JBTS), and Meckel-Gruber (MKS) syndromes
are autosomal-recessive ciliopathies presenting with cystic kidneys, retinal degeneration …

Disruption of intraflagellar transport in adult mice leads to obesity and slow-onset cystic kidney disease

JR Davenport, AJ Watts, VC Roper, MJ Croyle… - Current Biology, 2007 - cell.com
The assembly of primary cilia is dependant on intraflagellar transport (IFT), which mediates
the bidirectional movement of proteins between the base and tip of the cilium. In mice …

Nephronophthisis-associated ciliopathies

F Hildebrandt, W Zhou - Journal of the American society of …, 2007 - journals.lww.com
Nephronophthisis (NPHP), an autosomal recessive cystic kidney disease, represents the
most frequent genetic cause of end-stage kidney disease in the first three decades of life …

Dysfunctional cilia lead to altered ependyma and choroid plexus function, and result in the formation of hydrocephalus

B Banizs, MM Pike, CL Millican, WB Ferguson… - 2005 - journals.biologists.com
Cilia are complex organelles involved in sensory perception and fluid or cell movement.
They are constructed through a highly conserved process called intraflagellar transport (IFT) …

A change of heart: new roles for cilia in cardiac development and disease

L Djenoune, K Berg, M Brueckner… - Nature Reviews Cardiology, 2022 - nature.com
Although cardiac abnormalities have been observed in a growing class of human disorders
caused by defective primary cilia, the function of cilia in the heart remains an underexplored …

Polycystin-1, STAT6, and P100 function in a pathway that transduces ciliary mechanosensation and is activated in polycystic kidney disease

SH Low, S Vasanth, CH Larson, S Mukherjee… - Developmental cell, 2006 - cell.com
Primary cilia are implicated in the pathogenesis of autosomal-dominant polycystic kidney
disease (ADPKD), which results from defects in polycystin-1 (PC1), but the function of PC1 …

Acute kidney injury and aberrant planar cell polarity induce cyst formation in mice lacking renal cilia

V Patel, L Li, P Cobo-Stark, X Shao… - Human molecular …, 2008 - academic.oup.com
Polycystic kidney disease (PKD) is an inherited disorder that is characterized by the
accumulation of cysts in the renal parenchyma and progressive decline in renal function …

Type III adenylyl cyclase localizes to primary cilia throughout the adult mouse brain

GA Bishop, NF Berbari, J Lewis… - Journal of Comparative …, 2007 - Wiley Online Library
Solitary primary cilia project from nearly every cell type in the human body. These organelles
are considered to have important sensory and signaling functions. Although primary cilia …