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  • Parietal epithelial cells maintain the epithelial cell continuum forming Bowman's space in focal segmental glomerulosclerosis.

Parietal epithelial cells maintain the epithelial cell continuum forming Bowman's space in focal segmental glomerulosclerosis.

Disease models & mechanisms (2021-12-21)
Laura Miesen, Péter Bándi, Brigith Willemsen, Fieke Mooren, Thiago Strieder, Eva Boldrini, Vedran Drenic, Jennifer Eymael, Roy Wetzels, Johannes Lotz, Nick Weiss, Eric Steenbergen, Toin H van Kuppevelt, Merijn van Erp, Jeroen van der Laak, Nicole Endlich, Marcus J Moeller, Jack F M Wetzels, Jitske Jansen, Bart Smeets
ABSTRACT

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 pattern in Munich Wistar Frömter 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.

MATERIALS
Product Number
Brand
Product Description

Sigma-Aldrich
Monoclonal Anti-VSV Glycoprotein−Cy3 antibody produced in mouse, clone P5D4, purified immunoglobulin, buffered aqueous solution
Sigma-Aldrich
Anti-ANXA3 antibody produced in rabbit, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution