TRP channels as cellular sensors

DE Clapham - Nature, 2003 - nature.com
TRP channels are the vanguard of our sensory systems, responding to temperature, touch,
pain, osmolarity, pheromones, taste and other stimuli. But their role is much broader than …

Autosomal dominant polycystic kidney disease

VE Torres, PC Harris, Y Pirson - The Lancet, 2007 - thelancet.com
Autosomal dominant polycystic kidney disease is the most prevalent, potentially lethal,
monogenic disorder. It is associated with large interfamilial and intrafamilial variability, which …

Structure of the human PKD1-PKD2 complex

Q Su, F Hu, X Ge, J Lei, S Yu, T Wang, Q Zhou, C Mei… - Science, 2018 - science.org
INTRODUCTION Mutations in two genes, PKD1 and PKD2, are responsible for about 85 and
10% of all cases of autosomal dominant polycystic kidney disease (ADPKD), one of the most …

Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells

SM Nauli, FJ Alenghat, Y Luo, E Williams, P Vassilev… - Nature …, 2003 - nature.com
Several proteins implicated in the pathogenesis of polycystic kidney disease (PKD) localize
to cilia. Furthermore, cilia are malformed in mice with PKD with mutations in TgN737Rpw …

The structure of the polycystic kidney disease channel PKD2 in lipid nanodiscs

PS Shen, X Yang, PG DeCaen, X Liu, D Bulkley… - Cell, 2016 - cell.com
Summary The Polycystic Kidney Disease 2 (Pkd2) gene is mutated in autosomal dominant
polycystic kidney disease (ADPKD), one of the most common human monogenic disorders …

An introduction to TRP channels

IS Ramsey, M Delling, DE Clapham - Annu. Rev. Physiol., 2006 - annualreviews.org
▪ Abstract The aim of this review is to provide a basic framework for understanding the
function of mammalian transient receptor potential (TRP) channels, particularly as they have …

Transient receptor potential cation channels in disease

B Nilius, G Owsianik, T Voets… - Physiological …, 2007 - journals.physiology.org
The transient receptor potential (TRP) superfamily consists of a large number of cation
channels that are mostly permeable to both monovalent and divalent cations. The 28 …

Autosomal dominant polycystic kidney disease

PA Gabow - New England Journal of Medicine, 1993 - Mass Medical Soc
Autosomal dominant polycystic kidney disease is one of the most common hereditary
disorders, being 10 times more common than sickle cell disease, 15 times more common …

The primary cilium as the cell's antenna: signaling at a sensory organelle

V Singla, JF Reiter - science, 2006 - science.org
Almost every vertebrate cell has a specialized cell surface projection called a primary cilium.
Although these structures were first described more than a century ago, the full scope of their …

Functional polycystin-1 dosage governs autosomal dominant polycystic kidney disease severity

K Hopp, CJ Ward, CJ Hommerding… - The Journal of …, 2012 - Am Soc Clin Investig
Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations to PKD1 or
PKD2, triggering progressive cystogenesis and typically leading to end-stage renal disease …