Metabolism and mitochondria in polycystic kidney disease research and therapy

V Padovano, C Podrini, A Boletta… - Nature Reviews …, 2018 - nature.com
Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common,
potentially lethal, monogenic diseases and is caused predominantly by mutations in …

[HTML][HTML] Pathophysiology of colorectal peritoneal carcinomatosis: Role of the peritoneum

L Lemoine, P Sugarbaker… - World journal of …, 2016 - ncbi.nlm.nih.gov
Colorectal cancer (CRC) is the third most common cancer and the fourth most common
cause of cancer-related death worldwide. Besides the lymphatic and haematogenous routes …

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 …

The cell biology of polycystic kidney disease

HC Chapin, MJ Caplan - Journal of Cell Biology, 2010 - rupress.org
Polycystic kidney disease is a common genetic disorder in which fluid-filled cysts displace
normal renal tubules. Here we focus on autosomal dominant polycystic kidney disease …

Cyst formation and activation of the extracellular regulated kinase pathway after kidney specific inactivation of Pkd1

S Shibazaki, Z Yu, S Nishio, X Tian… - Human molecular …, 2008 - academic.oup.com
Polycystic kidney disease (ADPKD) results from failure of the kidney to properly maintain
three-dimensional structure after loss of either polycystin-1 or-2. Mice with kidney selective …

Mechanical stimuli induce cleavage and nuclear translocation of the polycystin-1 C terminus

V Chauvet, X Tian, H Husson… - The Journal of …, 2004 - Am Soc Clin Investig
Polycystin-1, which is encoded by a gene that is mutated in autosomal dominant polycystic
kidney disease (ADPKD), is involved in cell-matrix interactions as well as in ciliary signaling …

Cilia-like structures and polycystin-1 in osteoblasts/osteocytes and associated abnormalities in skeletogenesis and Runx2 expression

Z Xiao, S Zhang, J Mahlios, G Zhou… - Journal of Biological …, 2006 - ASBMB
We examined the osteoblast/osteocyte expression and function of polycystin-1 (PC1), a
transmembrane protein that is a component of the polycystin-2 (PC2)-ciliary mechano …

Altered trafficking and stability of polycystins underlie polycystic kidney disease

Y Cai, SV Fedeles, K Dong… - The Journal of …, 2014 - Am Soc Clin Investig
The most severe form of autosomal dominant polycystic kidney disease occurs in patients
with mutations in the gene (PKD1) encoding polycystin-1 (PC1). PC1 is a complex polytopic …

Polycystin-1: a master regulator of intersecting cystic pathways

SV Fedeles, AR Gallagher, S Somlo - Trends in molecular medicine, 2014 - cell.com
Autosomal dominant polycystic kidney disease (ADPKD) is the most common potentially
lethal monogenic disorder, with more than 12 million cases worldwide. The two causative …

[HTML][HTML] Cyclic AMP-mediated cyst expansion

DP Wallace - Biochimica et Biophysica Acta (BBA)-Molecular basis …, 2011 - Elsevier
In polycystic kidney disease (PKD), intracellular cAMP promotes cyst enlargement by
stimulating mural epithelial cell proliferation and transepithelial fluid secretion. The …