Polycystic kidney disease

C Bergmann, LM Guay-Woodford, PC Harris… - Nature reviews Disease …, 2018 - nature.com
Cystic kidneys are common causes of end-stage renal disease, both in children and in
adults. Autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive …

Cell adhesion by integrins

M Bachmann, S Kukkurainen… - Physiological …, 2019 - journals.physiology.org
Integrins are heterodimeric cell surface receptors ensuring the mechanical connection
between cells and the extracellular matrix. In addition to the anchorage of cells to the …

Renal plasticity revealed through reversal of polycystic kidney disease in mice

K Dong, C Zhang, X Tian, D Coman, F Hyder, M Ma… - Nature …, 2021 - nature.com
Initiation of cyst formation in autosomal dominant polycystic kidney disease (ADPKD) occurs
when kidney tubule cells are rendered null for either PKD1 or PKD2 by somatic 'second …

Extracellular matrix in kidney fibrosis: more than just a scaffold

RD Bülow, P Boor - Journal of Histochemistry & …, 2019 - journals.sagepub.com
Kidney fibrosis is the common histological end-point of progressive, chronic kidney diseases
(CKDs) regardless of the underlying etiology. The hallmark of renal fibrosis, similar to all …

Ciliary mechanisms of cyst formation in polycystic kidney disease

M Ma, AR Gallagher, S Somlo - Cold Spring Harbor …, 2017 - cshperspectives.cshlp.org
Autosomal-dominant polycystic kidney disease (ADPKD) is a disease of defective tissue
homeostasis resulting in active remodeling of nephrons and bile ducts to form fluid-filled …

[HTML][HTML] A polycystin-centric view of cyst formation and disease: the polycystins revisited

ACM Ong, PC Harris - Kidney international, 2015 - Elsevier
It is 20 years since the identification of PKD1, the major gene mutated in autosomal
dominant polycystic kidney disease (ADPKD), followed closely by the cloning of PKD2 …

The hallmarks of cancer: relevance to the pathogenesis of polycystic kidney disease

T Seeger-Nukpezah, DM Geynisman… - Nature Reviews …, 2015 - nature.com
Autosomal dominant polycystic kidney disease (ADPKD) is a progressive inherited disorder
in which renal tissue is gradually replaced with fluid-filled cysts, giving rise to chronic kidney …

Inflammation and fibrosis in polycystic kidney disease

CJ Song, KA Zimmerman, SJ Henke… - Kidney Development and …, 2017 - Springer
Polycystic kidney disease (PKD) is a commonly inherited disorder characterized by cyst
formation and fibrosis (Wilson, N Engl J Med 350: 151–164, 2004) and is caused by …

Renal regeneration: the role of extracellular matrix and current ECM‐based tissue engineered strategies

R Sobreiro‐Almeida, R Quinteira… - Advanced healthcare …, 2021 - Wiley Online Library
Natural extracellular matrices (ECM) are currently being studied as an alternative source for
organ transplantation or as new solutions to treat kidney injuries, which can evolve to end …

Polycystic kidney disease: Novel insights into polycystin function

L Luo, S Roy, L Li, M Ma - Trends in Molecular Medicine, 2023 - cell.com
Autosomal dominant polycystic kidney disease (ADPKD) is a life-threatening monogenic
disease caused by mutations in PKD1 and PKD2 that encode polycystin 1 (PC1) and …