ArticleAmerican journal of physiology. Cell physiology2010
Abnormalities in focal adhesion complex formation, regulation, and function in human autosomal recessive polycystic kidney disease epithelial cells.
Article in American journal of physiology. Cell physiology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 44 citations in OpenAlex.
- Fibrocystin/polyductin (FPC): new functional insights into ARPKD pathogenesis revealed by informatics, comparative genomics, and model systems.Pediatric nephrology (Berlin, Germany) · 2026Review
- Modeling Complex Developmental Disease: The Case of Polycystic Kidney Disease.Journal of developmental biology · 2026Review
- Focal adhesion kinase inhibition induces membrane accumulation of aquaporin-2 in renal epithelial cells by actin depolymerization and endocytosis inhibition.American journal of physiology. Renal physiology · 2025Article
- Reduced cell-substrate adhesion formation promotes cell migration inMolecular biology of the cell · 2025Article
- Defects of renal tubular homeostasis and cystogenesis in theiScience · 2024Article
- Primary URECs: a source to better understand the pathology of renal tubular epithelia in pediatric hereditary cystic kidney diseases.Orphanet journal of rare diseases · 2022Article
- Crizotinib: aseptic abscesses in multiple organs during treatment of EML4-ALK-positive NSCLC.Journal of cancer research and clinical oncology · 2021Article
- The carboxy-terminus of the human ARPKD protein fibrocystin can control STAT3 signalling by regulating SRC-activation.Journal of cellular and molecular medicine · 2020Article
- Use of patient derived urine renal epithelial cells to confirm pathogenicity of PKHD1 alleles.BMC nephrology · 2020Article
- Collecting duct cells show differential retinoic acid responses to acute versus chronic kidney injury stimuli.Scientific reports · 2020Article
- Fibrocystin Is Essential to Cellular Control of Adhesion and Epithelial Morphogenesis.International journal of molecular sciences · 2020Article
- Article
- Focal adhesion kinase (FAK) phosphorylation is a key regulator of embryonal rhabdomyosarcoma (ERMS) cell viability and migration.Journal of cancer research and clinical oncology · 2019Article
- Atmin modulates Pkhd1 expression and may mediate Autosomal Recessive Polycystic Kidney Disease (ARPKD) through altered non-canonical Wnt/Planar Cell Polarity (PCP) signalling.Biochimica et biophysica acta. Molecular basis of disease · 2019Article
- The association between autosomal dominant polycystic kidney disease and cancer.International urology and nephrology · 2019Review
- Development and Growth of the Avian Pectoralis Major (Breast) Muscle: Function of Syndecan-4 and Glypican-1 in Adult Myoblast Proliferation and Differentiation.Frontiers in physiology · 2017Review
- Gα12 is required for renal cystogenesis induced by Pkd1 inactivation.Journal of cell science · 2016Article
- Role of mechanotransduction in vascular biology: focus on thoracic aortic aneurysms and dissections.Circulation research · 2015Review
- Polycystin-1 and Gα12 regulate the cleavage of E-cadherin in kidney epithelial cells.Physiological genomics · 2015Article
- Epidermal growth factor-mediated proliferation and sodium transport in normal and PKD epithelial cells.Biochimica et biophysica acta · 2011Review
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
Abstract
Integrin-associated focal adhesion complex formation and turnover plays an essential role in directing interactions between epithelial cells and the extracellular matrix during organogenesis, leading to appropriate cell spreading, cell-matrix adhesion, and migration. Autosomal recessive polycystic kidney disease (ARPKD) is associated with loss of function of PKHD1-encoded protein fibrocystin-1 and is characterized by cystic dilation of renal collecting tubules (CT) in utero and loss of renal function in patients if they survive the perinatal period. Normal polycystin-1 (PC-1)/focal adhesion complex function is required for control of CT diameter during renal development, and abnormalities in these complexes have been demonstrated in human PC-1 mutant cystic cells. To determine whether loss of fibrocystin-1 was associated with focal adhesion abnormalities, ARPKD cells or normal age-matched human fetal (HF)CT cells in which fibrocystin-1 had been decreased by 85% by small interfering RNA inhibition were compared with normal HFCT. Accelerated attachment and spreading on collagen matrix and decreased motility of fibrocystin-1-deficient cells were associated with longer paxillin-containing focal adhesions, more complex actin-cytoskeletal rearrangements, and increased levels of total beta(1)-integrin, c-Src, and paxillin. Immunoblot analysis of adhesive cells using site-specific phospho-antibodies demonstrated ARPKD-associated loss of activation of focal adhesion kinase (FAK) by phosphorylation at its autophosphorylation site (Y397); accelerated FAK inhibition by phosphorylation at Y407, S843, and S910; as well as increased activation of c-Src at pY418. Paxillin coimmunoprecipitation analyses suggested that fibrocystin-1 was a component of the normal focal adhesion complex and that actin and fibrocystin-1 were lost from ARPKD complexes.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.