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 …

TRPP channels and polycystins

A Hofherr, M Köttgen - Transient Receptor Potential Channels, 2011 - Springer
The founding member of the TRPP family, TRPP2, was identified as one of the disease
genes causing autosomal dominant polycystic kidney disease (ADPKD). ADPKD is the most …

[HTML][HTML] A pathogenic C terminus-truncated polycystin-2 mutant enhances receptor-activated Ca2+ entry via association with TRPC3 and TRPC7

K Miyagi, S Kiyonaka, K Yamada, T Miki, E Mori… - Journal of Biological …, 2009 - ASBMB
Mutations in PKD2 gene result in autosomal dominant polycystic kidney disease (ADPKD).
PKD2 encodes polycystin-2 (TRPP2), which is a homologue of transient receptor potential …

The TRPP subfamily and polycystin-1 proteins

M Semmo, M Köttgen, A Hofherr - Mammalian Transient Receptor …, 2014 - Springer
It has been exciting times since the identification of polycystic kidney disease 1 (PKD1) and
PKD2 as the genes mutated in autosomal dominant polycystic kidney disease (ADPKD) …

[HTML][HTML] Extracellular loops are essential for the assembly and function of polycystin receptor-ion channel complexes

Z Salehi-Najafabadi, B Li, V Valentino, C Ng… - Journal of Biological …, 2017 - ASBMB
Polycystin complexes, or TRPP-PKD complexes, made of transient receptor potential
channel polycystin (TRPP) and polycystic kidney disease (PKD) proteins, play key roles in …

The multimeric structure of polycystin-2 (TRPP2): structural–functional correlates of homo-and hetero-multimers with TRPC1

P Zhang, Y Luo, B Chasan… - Human molecular …, 2009 - academic.oup.com
Abstract Polycystin-2 (PC2, TRPP2), the gene product of PKD2, whose mutations cause
autosomal dominant polycystic kidney disease (ADPKD), belongs to the superfamily of TRP …

The ion channel function of polycystin‐1 in the polycystin‐1/polycystin‐2 complex

Z Wang, C Ng, X Liu, Y Wang, B Li, P Kashyap… - EMBO …, 2019 - embopress.org
Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in PKD 1 or
PKD 2 gene, encoding the polycystic kidney disease protein polycystin‐1 and the transient …

[HTML][HTML] TRPP2 and autosomal dominant polycystic kidney disease

M Köttgen - Biochimica et Biophysica Acta (BBA)-Molecular Basis …, 2007 - Elsevier
Mutations in TRPP2 (polycystin-2) cause autosomal dominant polycystic kidney disease
(ADPKD), a common genetic disorder characterized by progressive development of fluid …

Subcellular localization and trafficking of polycystins

M Köttgen, G Walz - Pflügers Archiv, 2005 - Springer
Polycystin-2 is a member of the transient receptor potential (TRP) family of ion channels that
is mutated in autosomal dominant polycystic kidney disease. Although its function as a non …

Function and regulation of TRPP2 at the plasma membrane

L Tsiokas - American Journal of Physiology-Renal …, 2009 - journals.physiology.org
The vast majority (∼ 99%) of all known cases of autosomal dominant polycystic kidney
disease (ADPKD) are caused by naturally occurring mutations in two separate, but …