TY - JOUR
T1 - Parietal epithelial cells maintain the epithelial cell continuum forming Bowman`s space in focal segmental glomerulosclerosis
AU - Miesen, Laura
AU - Bándi, Péter
AU - Willemsen, Brigith
AU - Mooren, Fieke
AU - Strieder, Thiago
AU - Boldrini, Eva
AU - Drenic, Vedran
AU - Eymael, Jennifer
AU - Wetzels, Roy
AU - Lotz, Johannes
AU - Weiss, Nick
AU - Steenbergen, Eric
AU - van Kuppevelt, Toin H.
AU - van Erp, Merijn
AU - van der Laak, Jeroen
AU - Endlich, Nicole
AU - Moeller, Marcus J.
AU - Wetzels, Jack F. M.
AU - Jansen, Jitske
AU - Smeets, Bart
PY - 2022/3/14
Y1 - 2022/3/14
N2 - 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.
AB - 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.
M3 - Journal articles
SN - 1754-8403
VL - 2022
JO - DMM Disease Models and Mechanisms
JF - DMM Disease Models and Mechanisms
IS - Issue 3
ER -