Ciliopathies, the Role of Wnt-Signaling Pathway in the Course of Acute (Postinfectious) Glomerulonephritis: Literature Review and Own Observations

Abstract

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.

Keywords

• acute glomerulonephritis
• polycystic kidney disease
• ciliopathies
• Wnt-signaling pathway