TRP channels as potential drug targets

MM Moran - Annual review of pharmacology and toxicology, 2018 - annualreviews.org
The transient receptor potential (TRP) superfamily of channels comprises a diverse group of
cation channels. Four TRP channel subunits coassemble to form functional homo-or …

Primary ciliary dyskinesia: current state of the art

A Bush, R Chodhari, N Collins, F Copeland… - Archives of disease in …, 2007 - adc.bmj.com
Primary ciliary dyskinesia (PCD) is usually inherited as an autosomal recessive disorder and
presents with upper and lower respiratory tract infection, and mirror image arrangement in …

Proteomic analysis of a eukaryotic cilium

GJ Pazour, N Agrin, J Leszyk, GB Witman - The Journal of cell biology, 2005 - rupress.org
Cilia and flagella are widespread cell organelles that have been highly conserved
throughout evolution and play important roles in motility, sensory perception, and the life …

A genetic screen in zebrafish identifies cilia genes as a principal cause of cystic kidney

Z Sun, A Amsterdam, GJ Pazour, DG Cole, MS Miller… - 2004 - journals.biologists.com
Polycystic kidney disease (PKD) is a common human genetic illness. It is characterized by
the formation of multiple kidney cysts that are thought to result from over-proliferation of …

Polycystin-2 is an essential ion channel subunit in the primary cilium of the renal collecting duct epithelium

X Liu, T Vien, J Duan, SH Sheu, PG DeCaen… - elife, 2018 - elifesciences.org
Mutations in the polycystin genes, PKD1 or PKD2, results in Autosomal Dominant Polycystic
Kidney Disease (ADPKD). Although a genetic basis of ADPKD is established, we lack a …

INPP5E mutations cause primary cilium signaling defects, ciliary instability and ciliopathies in human and mouse

M Jacoby, JJ Cox, S Gayral, DJ Hampshire, M Ayub… - Nature …, 2009 - nature.com
The primary cilium is an antenna-like structure that protrudes from the cell surface of
quiescent/differentiated cells and participates in extracellular signal processing,,. Here, we …

TRPP2 and TRPV4 form a polymodal sensory channel complex

M Köttgen, B Buchholz, MA Garcia-Gonzalez… - The Journal of cell …, 2008 - rupress.org
The primary cilium has evolved as a multifunctional cellular compartment that decorates
most vertebrate cells. Cilia sense mechanical stimuli in various organs, but the molecular …

Think small: zebrafish as a model system of human pathology

JR Goldsmith, C Jobin - BioMed Research International, 2012 - Wiley Online Library
Although human pathologies have mostly been modeled using higher mammal systems
such as mice, the lower vertebrate zebrafish has gained tremendous attention as a model …

Renal cystic diseases: a review

M Bisceglia, CA Galliani, C Senger… - Advances in anatomic …, 2006 - journals.lww.com
This review aims to assist in the categorization of inherited, developmental, and acquired
cystic disease of the kidney as well as to provide a pertinent, up-to-date bibliography. The …

Glomerulocystic kidney disease in mice with a targeted inactivation of Wwtr1

Z Hossain, SM Ali, HL Ko, J Xu, CP Ng… - Proceedings of the …, 2007 - National Acad Sciences
Wwtr1 is a widely expressed 14-3-3-binding protein that regulates the activity of several
transcription factors involved in development and disease. To elucidate the physiological …