scholarly journals An inducible mouse model of podocin-mutation-related nephrotic syndrome

PLoS ONE ◽  
2017 ◽  
Vol 12 (10) ◽  
pp. e0186574 ◽  
Author(s):  
Mansoureh Tabatabaeifar ◽  
Tanja Wlodkowski ◽  
Ivana Simic ◽  
Helga Denc ◽  
Geraldine Mollet ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Mouse Model

scholarly journals Mild electrical stimulation with heat shock attenuates renal pathology in adriamycin-induced nephrotic syndrome mouse model

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Keisuke Teramoto ◽  
Yu Tsurekawa ◽  
Mary Ann Suico ◽  
Shota Kaseda ◽  
Kohei Omachi ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Electrical Stimulation ◽  
Heat Shock ◽  
Mouse Model ◽  
Akt Inhibitor ◽  
First Line ◽  
Renal Disorder ◽  
Apoptotic Effect ◽  
Pro Inflammatory Cytokines

Abstract Nephrotic syndrome (NS) is a renal disorder that is characterized by massive proteinuria, hypoalbuminemia and edema. One of the main causes of NS is focal segmental glomerulosclerosis (FSGS), which has extremely poor prognosis. Although steroids and immunosuppressants are the first line of treatment, some FSGS cases are refractory, prompting the need to find new therapeutic strategies. We have previously demonstrated that an optimized combination treatment of mild electrical stimulation (MES) and heat shock (HS) has several biological benefits including the amelioration of the pathologies of the genetic renal disorder Alport syndrome. Here, we investigated the effect of MES + HS on adriamycin (ADR)-induced NS mouse model. MES + HS suppressed proteinuria and glomerulosclerosis induced by ADR. The expressions of pro-inflammatory cytokines and pro-fibrotic genes were also significantly downregulated by MES + HS. MES + HS decreased the expression level of cleaved caspase-3 and the number of TUNEL-positive cells, indicating that MES + HS exerted anti-apoptotic effect. Moreover, MES + HS activated the Akt signaling and induced the phosphorylation and inhibition of the apoptotic molecule BAD. In in vitro experiment, the Akt inhibitor abolished the MES + HS-induced Akt-BAD signaling and anti-apoptotic effect in ADR-treated cells. Collectively, our study suggested that MES + HS modulates ADR-induced pathologies and has renoprotective effect against ADR-induced NS via regulation of Akt-BAD axis.

scholarly journals Plasminogen deficiency does not prevent sodium retention in a genetic mouse model of experimental nephrotic syndrome

2020 ◽  
Vol 231 (1) ◽  
Author(s):  
Mengyun Xiao ◽  
Bernhard N. Bohnert ◽  
Hande Aypek ◽  
Oliver Kretz ◽  
Florian Grahammer ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Mouse Model ◽  
Sodium Retention ◽  
Genetic Mouse Model ◽  
Plasminogen Deficiency

scholarly journals A Humanized Mouse Model of Idiopathic Nephrotic Syndrome Suggests a Pathogenic Role for Immature Cells

2007 ◽  
Vol 18 (10) ◽  
pp. 2732-2739 ◽  
Author(s):  
Anne-Laure Sellier-Leclerc ◽  
Arnaud Duval ◽  
Stéphanie Riveron ◽  
Marie-Alice Macher ◽  
Georges Deschenes ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Mouse Model ◽  
Idiopathic Nephrotic Syndrome ◽  
Humanized Mouse ◽  
Pathogenic Role ◽  
Humanized Mouse Model ◽  
Immature Cells

scholarly journals A deletion in the N-terminal polymerizing domain of laminin β2 is a new mouse model of chronic nephrotic syndrome

2020 ◽  
Vol 98 (1) ◽  
pp. 133-146 ◽  
Author(s):  
Steven D. Funk ◽  
Raymond H. Bayer ◽  
Karen K. McKee ◽  
Kazushi Okada ◽  
Hiroshi Nishimune ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Mouse Model ◽  
Laminin Β2

scholarly journals Ferulic Acid, anAngelica sinensis-Derived Polyphenol, Slows the Progression of Membranous Nephropathy in a Mouse Model

2012 ◽  
Vol 2012 ◽  
pp. 1-12 ◽  
Author(s):  
Chao-Wen Cheng ◽  
Wen-Liang Chang ◽  
Li-Cheng Chang ◽  
Chia-Chao Wu ◽  
Yuh-Feng Lin ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Mouse Model ◽  
Ferulic Acid ◽  
Membranous Nephropathy ◽  
Angelica Sinensis ◽  
Therapeutic Effects ◽  
Th2 Response ◽  
Adequate Treatment ◽  
Oxidative Stresses ◽  
Antiangiogenic Factors

Membranous nephropathy (MN) is a leading cause of adult nephrotic syndrome but lacks adequate treatment. Different extracts ofAngelica sinensis(AS) and one of its active compounds, ferulic acid (FA), were used to evaluate the therapeutic effects in a MN mouse model. The MN model was grouped into three subgroups: no treatment (N-T), treatment at induction of MN (Pre-T), and treatment after full-blown MN (Post-T). The results showed that the methanol (ME) layer of AS extract exhibited a therapeutic effect on MN-induced proteinuria. The ME layer-enriched compound, FA, improved the hypoalbuminemia, hyperlipidemia, and proteinuria in both Pre-T and Post-T groups. Ferulic acid also reduced the formation of oxidative protein products and increased the synthesis of antioxidant enzymes in groups Pre-T and Post-T. Regarding angiogenesis factors, the antiangiogenic factors in renal glomeruli were increased in group N-T, but, after FA treatment, only one of the antiangiogenic factors, thrombospondin-1, showed a significant decrease. Furthermore, the expression of Th2 predominant showed significant decrease in both Pre-T and Post-T groups when compared to that of N-T group. In summary, FA retarded the progression of MN, and the mechanisms involved the regulation of oxidative stresses, angiogenic and antiangiogenic factors, and attenuation of Th2 response.

Hereditary Nephrotic Syndrome with Progression to Renal Failure in a Mouse Model (ICGN Strain): Clinical Study

Nephron ◽  
1994 ◽  
Vol 68 (2) ◽  
pp. 239-244 ◽  
Author(s):  
Atsuo Ogura ◽  
Toshihiko Asano ◽  
Osamu Suzuki ◽  
Yoshie Yamamoto ◽  
Yoko Noguchi ◽  
...  
Keyword(s):  
Renal Failure ◽  
Nephrotic Syndrome ◽  
Clinical Study ◽  
Mouse Model

scholarly journals Nephrotic Syndrome and Subepithelial Deposits in a Mouse Model of Immune-Mediated Anti-Podocyte Glomerulonephritis

2011 ◽  
Vol 187 (6) ◽  
pp. 3218-3229 ◽  
Author(s):  
Catherine Meyer-Schwesinger ◽  
Silke Dehde ◽  
Philipp Klug ◽  
Jan U. Becker ◽  
Sabrina Mathey ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Mouse Model ◽  
Immune Mediated

Transgenic mouse model of kidney disease: Insertional inactivation of ubiquitously expressed gene leads to nephrotic syndrome

Cell ◽  
1990 ◽  
Vol 62 (3) ◽  
pp. 425-434 ◽  
Author(s):  
Hans Weiher ◽  
Tetsuo Noda ◽  
Douglas A. Gray ◽  
Arlene H. Sharpe ◽  
Rudolf Jaenisch
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Mouse Model ◽  
Transgenic Mouse ◽  
Transgenic Mouse Model ◽  
Insertional Inactivation

scholarly journals A Novel Model for Nephrotic Syndrome Reveals Associated Dysbiosis of the Gut Microbiome and Extramedullary Hematopoiesis

Cells ◽  
2021 ◽  
Vol 10 (6) ◽  
pp. 1509
Author(s):  
Jasmin I. Maier ◽  
Manuel Rogg ◽  
Martin Helmstädter ◽  
Alena Sammarco ◽  
Gerd Walz ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Mouse Model ◽  
Gut Microbiome ◽  
Extramedullary Hematopoiesis ◽  
Glomerular Sclerosis ◽  
Protein Loss ◽  
Adult Mice ◽  
Focal Segmental Glomerular Sclerosis ◽  
Significant Enrichment

Glomerular kidney disease causing nephrotic syndrome is a complex systemic disorder and is associated with significant morbidity in affected patient populations. Despite its clinical relevance, well-established models are largely missing to further elucidate the implications of uncontrolled urinary protein loss. To overcome this limitation, we generated a novel, inducible, podocyte-specific transgenic mouse model (Epb41l5fl/fl*Nphs1-rtTA-3G*tetOCre), developing nephrotic syndrome in adult mice. Animals were comprehensively characterized, including microbiome analysis and multiplexed immunofluorescence imaging. Induced knockout mice developed a phenotype consistent with focal segmental glomerular sclerosis (FSGS). Although these mice showed hallmark features of severe nephrotic syndrome (including proteinuria, hypoalbuminemia and dyslipidemia), they did not exhibit overt chronic kidney disease (CKD) phenotypes. Analysis of the gut microbiome demonstrated distinct dysbiosis and highly significant enrichment of the Alistipes genus. Moreover, Epb41l5-deficient mice developed marked organ pathologies, including extramedullary hematopoiesis of the spleen. Multiplex immunofluorescence imaging demonstrated red pulp macrophage proliferation and mTOR activation as driving factors of hematopoietic niche expansion. Thus, this novel mouse model for adult-onset nephrotic syndrome reveals the significant impact of proteinuria on extra-renal manifestations, demonstrating the versatility of this model for nephrotic syndrome-related research.

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