期刊
DISEASE MODELS & MECHANISMS
卷 15, 期 3, 页码 -出版社
COMPANY BIOLOGISTS LTD
DOI: 10.1242/dmm.046342
关键词
Parietal epithelial cells; Focal segmental glomerulosclerosis; Epithelial continuum; Bowman's space; Munich Wister Fromter rat
资金
- Nierstichting (Dutch Kidney Foundation) [14A3D104]
- Nederlandse Organisatie voor Wetenschappelijk Onderzoek (Netherlands Organization for Scientific Research) NWO VIDI grant [016.156.363]
In focal segmental glomerulosclerosis (FSGS), the continuity of glomerular epithelial cells is disrupted, but non-sclerotic segments remain protected. Studies have shown that in FSGS, parietal epithelial cells (PECs) can cover the sclerotic lesions and restore the continuity of glomerular epithelial cells.
In the glomerulus, Bowman's space is formed by a continuum of glomerular epithelial cells. In focal segmental glomerulosclerosis (FSGS), glomeruli show segmental scarring, a result of activated parietal epithelial cells (PECs) invading the glomerular tuft. The segmental scars interrupt the epithelial continuum. However, non-sclerotic segments seem to be preserved even in glomeruli with advanced lesions. We studied the histology of the segmental patter n in Munich Wistar Fro'' mter rats, a model for secondary FSGS. Our results showed that matrix layers lined with PECs cover the sclerotic lesions. These PECs formed contacts with podocytes of the uninvolved tuft segments, restoring the epithelial continuum. Formed Bowman's spaces were still connected to the tubular system. In biopsies of patients with secondary FSGS, we also detected matrix layers formed by PECs, separating the uninvolved from the sclerotic glomerular segments. PECs have a major role in the formation of glomerulosclerosis; we show here that in FSGS they also restore the glomerular epithelial cell continuum that surrounds Bowman's space. This process may be beneficial and indispensable for glomerular filtration in the uninvolved segments of sclerotic glomeruli.
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