[HTML][HTML] A polycystin-centric view of cyst formation and disease: the polycystins revisited

ACM Ong, PC Harris - Kidney international, 2015 - Elsevier
It is 20 years since the identification of PKD1, the major gene mutated in autosomal
dominant polycystic kidney disease (ADPKD), followed closely by the cloning of PKD2 …

Structural biology of TRP channels

UA Hellmich, R Gaudet - … Potential (TRP) Cation Channels: Volume II, 2014 - Springer
Membrane proteins remain challenging targets for structural biologists, despite recent
technical developments regarding sample preparation and structure determination. We …

Molecular architecture and subunit organization of TRPA1 ion channel revealed by electron microscopy

TL Cvetkov, KW Huynh, MR Cohen… - Journal of Biological …, 2011 - ASBMB
Transient receptor potential ankyrin 1 (TRPA1) is a non-selective ion channel, which is
expressed in nociceptor sensory neurons and transduces chemical, inflammatory, and …

Polycystin-2 activation by inositol 1, 4, 5-trisphosphate-induced Ca2+ release requires its direct association with the inositol 1, 4, 5-trisphosphate receptor in a …

E Sammels, B Devogelaere, D Mekahli… - Journal of biological …, 2010 - ASBMB
Autosomal dominant polycystic kidney disease is characterized by the loss-of-function of a
signaling complex involving polycystin-1 and polycystin-2 (TRPP2, an ion channel of the …

Determinants of TRPV4 activity following selective activation by small molecule agonist GSK1016790A

M Jin, Z Wu, L Chen, J Jaimes, D Collins, ET Walters… - PloS one, 2011 - journals.plos.org
TRPV4 (Transient Receptor Potential Vanilloid 4) channels are activated by a wide range of
stimuli, including hypotonic stress, non-noxious heat and mechanical stress and some small …

Structure and function of polycystins: insights into polycystic kidney disease

D Douguet, A Patel, E Honoré - Nature Reviews Nephrology, 2019 - nature.com
Mutations in the polycystins PC1 or PC2 cause autosomal dominant polycystic kidney
disease (ADPKD), which is characterized by the formation of fluid-filled renal cysts that …

Function and regulation of TRPP2 ion channel revealed by a gain-of-function mutant

M Arif Pavel, C Lv, C Ng, L Yang… - Proceedings of the …, 2016 - National Acad Sciences
Mutations in polycystin-1 and transient receptor potential polycystin 2 (TRPP2) account for
almost all clinically identified cases of autosomal dominant polycystic kidney disease …

Structural biology of TRP channels

M Li, Y Yu, J Yang - Transient Receptor Potential Channels, 2011 - Springer
Structural studies on TRP channels, while limited, are poised for a quickened pace and
rapid expansion. As of yet, no high-resolution structure of a full length TRP channel exists …

Polycystin-1, 2, and STIM1 Interact with IP3R to Modulate ER Ca2+ Release through the PI3K/Akt Pathway

NG Santoso, L Cebotaru, WB Guggino - Cellular Physiology and …, 2011 - karger.com
Dysregulation of Ca2+ signaling and homeostasis has been linked to the development of
ADPKD through aberrant functioning of the polycystins. In this study, we investigated the role …

Structure of the EF-hand domain of polycystin-2 suggests a mechanism for Ca2+-dependent regulation of polycystin-2 channel activity

ET Petri, A Ćelić, SD Kennedy… - Proceedings of the …, 2010 - National Acad Sciences
The C-terminal cytoplasmic tail of polycystin-2 (PC2/TRPP2), a Ca2+-permeable channel, is
frequently mutated or truncated in autosomal dominant polycystic kidney disease. We have …