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

壁层上皮细胞维持局灶节段性肾小球硬化症中形成鲍曼氏腔的上皮细胞连续体

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作者: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 J

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.

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