Ciliopathies, the Role of Wnt-Signaling Pathway in the Course of Acute (Postinfectious) Glomerulonephritis: Literature Review and Own Observations
Primary cilia — the cellular organelles derived from cytoplasmic cell membrane of almost any type. They play a role in the perception and the transfer of mechanical and chemical signals from the surface of cells, orientation of division plane, organogenesis, etc. Mentioned features are implemented due to the large number of proteins, receptors, signal transduction pathways. Mutations and deregulation of these components can have a wide range of phenotypic effects united by a common name — ciliopaties, which include, in particular, cystic lesions of kidney parenchyma. Among other signaling cascades associated with primary cilia, Wnt-signaling pathway is responsible for nephrogenesis and response of the kidneys to damaging factors. There is described a clinical case of acute (postinfectious) glomerulonephritis in patient Kh., aged 20 years, with cystic dilation of kidney tubules that were diagnosed histologically. In the article, based on the recent researches in the ciliopathy field, we assume the possible molecular mechanisms responsible both for the formation of a cyst in the renal tubular system, and for progressive fatal course of glomerulonephritis in this case.
Full Text:PDF (Українська)
Habbig S., Liebau M. Ciliopathies — from rare inherited cystic kidney diseases to basic cellular function // Mol. Cell Pediatr. — 2015. — 2(1).
Wang S., Dong Z. Primary cilia and kidney injury: current research status and future perspectives // AJP: Renal Physiology. — 2013. — 305(8). – Р. F1085-F1098
Basten S., Giles R. Functional aspects of primary cilia in signaling, cell cycle and tumorigenesis // Cilia. — 2013. — 2(1). — Р. 6.
Waters A., Beales P. Ciliopathies: an expanding disease spectrum // Pediatr. Nephrol. — 2011. — 26(7). — Р. 1039-1056.
Lancaster M., Gleeson J. Cystic kidney disease: the role of Wnt signaling // Trends in Molecular Medicine. — 2010. — 16(8). — Р. 349-360.
Kawakami T., Ren S., Duffield J. Wnt signalling in kidney diseases: dual roles in renal injury and repair // J. Pathol. — 2012. — 229(2). — Р. 221-231.
Goggolidou P. Wnt and planar cell polarity signaling in cystic renal disease // Organogenesis. — 2013. — 10(1). — Р. 86-95.
Dai C., Stolz D.B., Kiss L.P. et al. Wnt/β-catenin signaling promotes podocyte dysfunction and albuminuria // J. Am. Soc. Nephrol. — 2009. — 20. — 1997-2008.
Kato H., Gruenwald A., Suh J.H. et al. Wnt/β-catenin pathway in podocytes integrates cell adhesion, differentiation, and survival // J. Biol. Chem. — 2011. — 286. — 26003-26015.
Heikkila E., Juhila J., Lassila M. et al. β-Catenin mediates adriamycin-induced albuminuria and podocyte injury in adult mouse kidneys // Nephrol. Dial Transpl. — 2010. — 25. — 2437-2446.
Copyright (c) 2016 KIDNEYS
This work is licensed under a Creative Commons Attribution 4.0 International License.
© "Publishing House "Zaslavsky", 1997-2019