AI Article Synopsis
- Focal segmental glomerulosclerosis (FSGS) is a major cause of nephrotic syndrome that resists steroid treatment, often leading to kidney failure, with spontaneous recovery being uncommon.
- The study reveals that the basic leucine zipper transcription factor, MafB, is crucial for podocyte function and is decreased in patients with FSGS; mice lacking MafB specifically in podocytes developed FSGS and severe proteinuria.
- Overexpressing MafB in podocytes of transgenic mice was found to reduce adriamycin-induced FSGS symptoms, suggesting MafB as a potential new target for FSGS therapies.
Article Abstract
Focal segmental glomerulosclerosis (FSGS) is a common cause of steroid-resistant nephrotic syndrome. Spontaneous remission of FSGS is rare and steroid-resistant FSGS frequently progresses to renal failure. Many inheritable forms of FSGS have been described, caused by mutations in proteins that are important for podocyte function. Here, we show that a basic leucine zipper transcription factor, MafB, protects against FSGS. MAFB expression was found to be decreased in the podocytes of patients with FSGS. Moreover, conditional podocyte-specific MafB-knockout mice developed FSGS with massive proteinuria accompanied by depletion of the slit diaphragm-related proteins (Nphs1 and Magi2), and the podocyte-specific transcription factor Tcf21. These findings indicate that MafB plays a crucial role in the pathogenesis of FSGS. Consistent with this, adriamycin-induced FSGS and attendant proteinuria were ameliorated by MafB overexpression in the podocytes of MafB podocyte-specific transgenic mice. Thus, MafB could be a new therapeutic target for FSGS.
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| http://dx.doi.org/10.1016/j.kint.2020.02.038 | DOI Listing |
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