Abstract

SCHIMPF, Judith  and  KRAMANN, Rafael. Cellular origin and regulation of kidney fibrosis. Port J Nephrol Hypert [online]. 2018, vol.32, n.3, pp.279-282. ISSN 0872-0169.

Myofibroblasts take a key position as fibrosis driving, matrix secreting cells in kidney fibrosis and are thought to be important therapeutic targets in chronic kidney disease (CKD). However, their origin and activation pattern have been discussed for many years and are still partly unclear. Recently, Gli1+ cells, which reside in the perivascular niche, have been identified as progenitors of fibrosis-causing myofibroblasts. However, Gli1+ cells only account for about 50% of the myofibroblast population and are predominantly located in the kidney medulla. Nevertheless, the data suggests that Gli1+ cells are an important therapeutic target in kidney fibrosis since genetic ablation of these cells significantly ameliorates kidney fibrosis in rodents. Other potential sources of myofibroblasts in the kidney are circulating bone-marrow derived cells, endothelium and epithelium. The current review will discuss the cellular origin of myofibroblasts and potential mechanisms of myofibroblast activation driving fibrosis and CKD.

Keywords : kidney fibrosis; myofibroblasts; Gli1; Hedgehog.

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