Fibrocystin is essential to cellular control of adhesion and epithelial morphogenesis

WH Ziegler, B Soetje, LP Marten, J Wiese… - International journal of …, 2020 - mdpi.com
Mutations of the Pkhd1 gene cause autosomal recessive polycystic kidney disease
(ARPKD). Pkhd1 encodes fibrocystin/polyductin (FPC), a ciliary type I membrane protein of …

Inhibition of Pkhd1 Impairs Tubulomorphogenesis of Cultured IMCD Cells

W Mai, D Chen, T Ding, I Kim, S Park… - Molecular biology of …, 2005 - Am Soc Cell Biol
Fibrocystin/polyductin (FPC), the gene product of PKHD1, is responsible for autosomal
recessive polycystic kidney disease (ARPKD). This disease is characterized by …

Fibrocystin/polyductin modulates renal tubular formation by regulating polycystin-2 expression and function

I Kim, Y Fu, K Hui, G Moeckel, W Mai, C Li… - Journal of the …, 2008 - journals.lww.com
Autosomal recessive polycystic kidney disease is caused by mutations in PKHD1, which
encodes the membrane-associated receptor-like protein fibrocystin/polyductin (FPC). FPC …

Polycystic kidney disease protein fibrocystin localizes to the mitotic spindle and regulates spindle bipolarity

J Zhang, M Wu, S Wang, JV Shah… - Human molecular …, 2010 - academic.oup.com
Autosomal recessive polycystic kidney disease (ARPKD) is a significant hereditary renal
disease occurring in infancy and childhood, which presents with greatly enlarged echogenic …

Cilia and polycystic kidney disease

M Ma - Seminars in Cell & Developmental Biology, 2021 - Elsevier
Polycystic kidney disease (PKD), comprising autosomal dominant polycystic kidney disease
(ADPKD) and autosomal recessive polycystic kidney d isease (ARPKD), is characterized by …

Divergent function of polycystin 1 and polycystin 2 in cell size regulation

A Viau, F Kotsis, C Boehlke, S Braeg, M Klein… - Biochemical and …, 2020 - Elsevier
Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in PKD1 or
PKD2, the genes encoding polycystin 1 (PC1) and polycystin 2 (PC2), respectively. PC1 and …

Abnormalities in focal adhesion complex formation, regulation, and function in human autosomal recessive polycystic kidney disease epithelial cells

S Israeli, K Amsler, N Zheleznova… - American Journal of …, 2010 - journals.physiology.org
Integrin-associated focal adhesion complex formation and turnover plays an essential role in
directing interactions between epithelial cells and the extracellular matrix during …

Loss of cilia does not slow liver disease progression in mouse models of autosomal recessive polycystic kidney disease

AR Gallagher, S Somlo - Kidney360, 2020 - journals.lww.com
Autosomal dominant polycystic kidney disease (ADPKD) is a hereditary disorder
characterized by abnormal cellular proliferation, cyst fluid accumulation, extracellular matrix …

Polycystin-1 induces cell migration by regulating phosphatidylinositol 3-kinase-dependent cytoskeletal rearrangements and GSK3β-dependent cell–cell mechanical …

M Boca, L D'Amato, G Distefano… - Molecular biology of …, 2007 - Am Soc Cell Biol
Polycystin-1 (PC-1) is a large plasma-membrane receptor encoded by the PKD1 gene
mutated in autosomal dominant polycystic kidney disease (ADPKD). Although the disease is …

Fibrocystin/polyductin, found in the same protein complex with polycystin-2, regulates calcium responses in kidney epithelia

S Wang, J Zhang, SM Nauli, X Li… - … and cellular biology, 2007 - Taylor & Francis
Recent evidence suggests that fibrocystin/polyductin (FPC), polycystin-1 (PC1), and
polycystin-2 (PC2) are all localized at the plasma membrane and the primary cilium, where …