Identification and characterization of a novel polycystin family member, polycystin-L2, in mouse and human: sequence, expression, alternative splicing, and …

L Guo, TH Schreiber, S Weremowicz, CC Morton… - Genomics, 2000 - Elsevier
Polycystins-1,-2,-L, and-REJ are the four known members of the polycystin family of proteins.
In this study, we describe a fifth member of the family, polycystin-L2, encoded by PKD2L2 in …

[HTML][HTML] Polycystin-1 transforms the cAMP growth-responsive phenotype of M-1 cells

M Sutters, T Yamaguchi, RL Maser, BS Magenheimer… - Kidney international, 2001 - Elsevier
Polycystin-1 transforms the cAMP growth-responsive phenotype of M-1 cells. Background
Polycystic kidney disease (PKD) is characterized by the abnormal proliferation of tubular …

[HTML][HTML] The polycystin-1 C-type lectin domain binds carbohydrate in a calcium-dependent manner, and interacts with extracellular matrix proteins in vitro

BS Weston, C Bagnéris, RG Price, JL Stirling - Biochimica Et Biophysica …, 2001 - Elsevier
Mutations in the PKD1 gene are responsible for 85% of cases of autosomal dominant
polycystic kidney disease (ADPKD). This gene encodes a large membrane associated …

The polycystic kidney disease proteins, polycystin-1, polycystin-2, polaris, and cystin, are co-localized in renal cilia

BK Yoder, X Hou… - Journal of the American …, 2002 - journals.lww.com
Recent evidence has suggested an association between structural and/or functional defects
in the primary apical cilium of vertebrate epithelia and polycystic kidney disease (PKD). In …

The cytoplasmic C-terminal fragment of polycystin-1 regulates a Ca2+-permeable cation channel

DH Vandorpe, MN Chernova, L Jiang, LK Sellin… - Journal of Biological …, 2001 - ASBMB
The cytoplasmic C-terminal portion of the polycystin-1 polypeptide (PKD1 (1–226)) regulates
several important cell signaling pathways, and its deletion suffices to cause autosomal …

Constitutive activation of G-proteins by polycystin-1 is antagonized by polycystin-2

P Delmas, H Nomura, X Li, M Lakkis, Y Luo… - Journal of Biological …, 2002 - ASBMB
Polycystin-1 (PC1), a 4,303-amino acid integral membrane protein of unknown function,
interacts with polycystin-2 (PC2), a 968-amino acid α-type channel subunit. Mutations in …

Polycystin-1 maturation requires polycystin-2 in a dose-dependent manner

VG Gainullin, K Hopp, CJ Ward… - The Journal of …, 2015 - Am Soc Clin Investig
Autosomal dominant polycystic kidney disease (ADPKD) is a common inherited
nephropathy responsible for 4%–10% of end-stage renal disease cases. Mutations in the …

Molecular basis of autosomal dominant polycystic kidney disease

L Al-Bhalal, M Akhtar - Advances in anatomic pathology, 2005 - journals.lww.com
Autosomal dominant polycystic kidney disease (ADPKD) is a serious, life-threatening
genetic disease in which extensive epithelial-lined cysts develop in the kidneys and, to a …

[HTML][HTML] New insights into the molecular pathophysiology of polycystic kidney disease

NS Murcia, WE Sweeney Jr, ED Avner - Kidney international, 1999 - Elsevier
New insights into the molecular pathophysiology of polycystic kidney disease. Polycystic
kidney diseases are characterized by the progressive expansion of multiple cystic lesions …

The heteromeric PC-1/PC-2 polycystin complex is activated by the PC-1 N-terminus

K Ha, M Nobuhara, Q Wang, RV Walker, F Qian… - Elife, 2020 - elifesciences.org
Mutations in the polycystin proteins, PC-1 and PC-2, result in autosomal dominant polycystic
kidney disease (ADPKD) and ultimately renal failure. PC-1 and PC-2 enrich on primary cilia …