Glomerular diseases最新文献

{"title":"Minimal Change Podocytopathy with Coexistent Thin Glomerular Basement Membrane following Exposure to Semaglutide.","authors":"Karthik Kovvuru, Swetha Rani Kanduri, Johnathon Phillips, Juan Carlos Velez","doi":"10.1159/000543357","DOIUrl":"10.1159/000543357","url":null,"abstract":"<p><strong>Introduction: </strong>Glucagon-like peptide-1 receptor agonists (GLP-1RAs) are FDA-approved for weight loss and are increasingly prescribed for patients with obesity and type 2 diabetes mellitus. Multiple studies have demonstrated desirable renal and cardiovascular benefits from these novel agents. So far, a few case reports of acute tubular injury and acute interstitial nephritis have been reported with GLP-1RA. Podocytopathies in association with semaglutide are rare. In this case report, we present a case of nephrotic syndrome that developed after exposure to semaglutide and propose potential pathophysiological mechanisms underlying this rare renal complication.</p><p><strong>Case presentation: </strong>Herein, we report a novel case of a 43-year-old female who was evaluated in the nephrology clinic for abrupt onset of bilateral lower extremity edema and foamy urine, a few weeks after exposure to semaglutide. She was diagnosed with nephrotic syndrome and subsequently underwent a kidney biopsy, which revealed features suggestive of minimal change disease, along with coexistent thin basement membrane disease.</p><p><strong>Conclusion: </strong>GLP-1RAs have been increasingly prescribed due to their proven pleiotropic benefits, including improvements in albuminuria, glycemic control, weight loss, and cardioprotective effects. Despite the considerable benefits of GLP-1RAs, it is essential to recognize novel side effects.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"103-108"},"PeriodicalIF":0.0,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11875556/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143544779","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sodium-Glucose Cotransporter 2 Inhibitors in Diabetic Kidney Disease and beyond.","authors":"Zein Alabdin Hannouneh, C Elena Cervantes, Mohamad Hanouneh, Mohamed G Atta","doi":"10.1159/000543685","DOIUrl":"10.1159/000543685","url":null,"abstract":"<p><strong>Background: </strong>Sodium-glucose cotransporter 2 inhibitors (SGLT2is) have significantly impacted the management of diabetic kidney disease (DKD) and heart failure (HF), providing benefits beyond glycemic control. This review examines the mechanisms through which SGLT2is provide renal and cardiovascular protection and assesses their clinical efficacy.</p><p><strong>Summary: </strong>By inducing glucosuria and natriuresis, SGLT2is alleviate multiple complications induced by chronic hyperglycemia. Moreover, SGLT2is reduce albuminuria, improve tubular function, and modulate erythropoiesis. Additionally, they mitigate inflammation and fibrosis by decreasing oxidative stress and downregulating proinflammatory pathways. Clinical trials have demonstrated significant reductions in renal and cardiovascular events among patients with type 2 diabetes mellitus. A comprehensive review of the literature was conducted through PubMed, highlighting the effects of SGLT2is and the results of major clinical trials involving SGLT2is.</p><p><strong>Key messages: </strong>SGLT2is play a crucial role in the management of DKD and HF by addressing multiple pathogenic pathways. Currently, SGLT2is are included in clinical guidelines for DKD and HF management, and their benefits extend to nondiabetic populations. Further research is needed to explore SGLT2is' multifaceted mechanisms and potential applications across diverse patient populations and different disease etiologies.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"119-132"},"PeriodicalIF":0.0,"publicationDate":"2025-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11906174/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143627027","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Genetic and Iatrogenic Defects in Peripheral Tolerance Associated with Anti-Nephrin Antibody-Associated Minimal Change Disease.","authors":"J Ashley Jefferson, Karin Chen, Sangeeta Hingorani, A Bilal Malik, Scott S Tykodi, Keith H Keller, Yuan Huang, Kelly D Smith, Robyn C Reed, Astrid Weins, Shreeram Akilesh","doi":"10.1159/000543334","DOIUrl":"10.1159/000543334","url":null,"abstract":"<p><strong>Introduction: </strong>Minimal change disease (MCD) is a common cause of nephrotic syndrome in children and adults. Immune dysregulation is a contributor, but the relative roles of individual components of the immune system in MCD pathogenesis remain unclear.</p><p><strong>Case presentation: </strong>Here, we present 2 patients with defects in immune tolerance mechanisms that developed MCD associated with anti-nephrin antibodies. The first patient had a pathogenic deletion in <i>FOXP3</i>, leading to reduced regulatory T cells. Serum could not be obtained from this patient during the active phase of MCD to directly establish the presence of anti-nephrin antibodies. However, this patient demonstrated IgG dusting over podocyte cell bodies by immunofluorescence microscopy, as well as colocalization of IgG with nephrin in confocal microscopy. The second patient developed MCD in the context of immune checkpoint inhibitor treatment for metastatic carcinoma. Anti-nephrin antibodies were detected in this patient during active disease. The patient's kidney biopsy also showed evidence of binding of anti-nephrin antibodies within the glomeruli.</p><p><strong>Conclusion: </strong>These cases demonstrate that genetic and iatrogenic mechanisms of breakdown in peripheral tolerance can lead to MCD.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"74-83"},"PeriodicalIF":0.0,"publicationDate":"2025-01-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11845169/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143484308","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"5th International Renal Pathology Conference in Zagreb, Croatia: Meeting Report.","authors":"Petar Šenjug, Danica Galešić Ljubanović","doi":"10.1159/000542726","DOIUrl":"10.1159/000542726","url":null,"abstract":"","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"19-25"},"PeriodicalIF":0.0,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11842037/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143484965","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mitigated Clinical Course of Crescentic Glomerulonephritis with Positive Anti-Glomerular Basement Membrane Antibodies in a 14-Year-Old Girl.","authors":"Noortje M van der Meulen, Michiel J S Oosterveld, Marjolijn S W Quaak, Ester M M van Leeuwen, Joris J T H Roelofs, Rik Westland","doi":"10.1159/000543339","DOIUrl":"10.1159/000543339","url":null,"abstract":"<p><strong>Introduction: </strong>Anti-glomerular basement membrane (anti-GBM) glomerulonephritis is associated with severe kidney failure and high morbidity and is exceedingly rare in children. The few pediatric studies published about this condition report dependency of kidney replacement therapy at presentation and partial or complete response in kidney function in a minority of cases.</p><p><strong>Case presentation: </strong>We describe the case of a 14-year-old girl with crescentic glomerulonephritis based on double positive anti-GBM and myeloperoxidase-antineutrophil cytoplasmic antibodies with a mitigated clinical course presenting with fatigue, anemia, and an estimated glomerular filtration rate range between 34 and 42 mL/min/1.73 m². Response to treatment with daily therapeutic plasma exchanges, corticosteroids, and oral cyclophosphamide was prompt.</p><p><strong>Conclusion: </strong>This ultrarare presentation highlights the need to consider determining anti-GBM antibodies and/or obtaining a kidney biopsy even in children with less severe presentations of unexplained glomerulonephritis and underlines the clinical treatment dilemma in this disease for children due to the potential long-term sequelae.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"68-73"},"PeriodicalIF":0.0,"publicationDate":"2025-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11845167/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143484327","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Practical Management of ANCA-Associated Vasculitis: A Clinician's Perspective.","authors":"Hannah L Stacey, Lucy Francis, Rona M Smith, Rachel B Jones","doi":"10.1159/000543159","DOIUrl":"10.1159/000543159","url":null,"abstract":"<p><strong>Background: </strong>Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis can be a life-threatening condition, characterized by necrotizing inflammation of small blood vessels. Major organ involvement, most commonly kidney and lung disease, is associated with significant morbidity and mortality. Intensive early immunosuppressive therapy is the cornerstone of management and has transformed ANCA-associated vasculitis (AAV) into a chronic relapsing condition. Remission induction with tapering glucocorticoids in combination with cyclophosphamide or rituximab is the standard of care for severe disease. Avacopan, an oral C5aR1 antagonist, has been approved for remission induction and helps minimize glucocorticoid exposure. Plasma exchange should be considered for severe kidney or life-threatening disease. Lower dose glucocorticoid induction regimens can be used without compromising remission rates. Remission maintenance therapy is recommended, and rituximab is usually first line over azathioprine. Mycophenolate mofetil (MMF) or methotrexate with low-dose glucocorticoids are third-line options. Immunosuppression-associated infection risk remains a concern, both during acute presentations and in the long term, highlighted by the impact of rituximab on humoral immunity and vaccine response during the COVID-19 pandemic. There remains an ongoing need for therapies that induce rapid remission and optimize kidney recovery while minimizing infection risk. Clinical trials are evaluating newer therapeutic options. Due to increasing treatment options, management should be individualized, balancing effective immunosuppression against comorbidities and frailty.</p><p><strong>Summary: </strong>This review focuses on the treatment decision pathways for clinicians and patients in the management of severe AAV (granulomatosis with polyangiitis and microscopic polyangiitis). Key clinical trials, predictors of outcome, novel therapeutics, and practical steps to mitigate infection risk are discussed.</p><p><strong>Key messages: </strong>Immunosuppression regimens have been refined due to emerging evidence from clinical trials. Rituximab, avacopan, and reduced-dose glucocorticoid schedules have been the focus of recent studies. Infections and immunosuppression-induced immunodeficiency must be considered when determining individualized treatment.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"84-102"},"PeriodicalIF":0.0,"publicationDate":"2024-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11845170/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143484637","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"ANCA-Associated Glomerulonephritis: Diagnosis and Therapy Proceedings of the Henry Shavelle Lectureship.","authors":"Vanja Ivković, Martin Windpessl, Ilay Berke, Duvuru Geetha, Jasper Callemeyn, Sayna Norouzi, Ingeborg M Bajema, Andreas Kronbichler","doi":"10.1159/000542925","DOIUrl":"10.1159/000542925","url":null,"abstract":"<p><strong>Background: </strong>Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) frequently affects the kidney. Glomerulonephritis (GN) in AAV, ANCA-GN, not only dictates therapeutic decisions but is also of relevance for overall survival influencing the risk of cardiovascular disease and serious infections.</p><p><strong>Summary: </strong>A diagnosis of ANCA-GN includes laboratory investigations including urinalysis and a thorough assessment of potential organ involvement. A kidney biopsy can be performed to ascertain the diagnosis but has an additional prognostic relevance and tools have been established to predict long-term kidney survival. Experimental biomarkers indicating kidney inflammation include urinary soluble CD163 and the presence of urinary T cells. Therapeutic options are refined and some of these therapies, such as the added value of performing plasma exchange, are the matter of controversial discussions. Safe reduction of cumulative exposure to glucocorticoids and eventually the use of avacopan to substantially reduce glucocorticoid exposure has been implemented in most centers. In the remission of maintenance, the optimal duration of therapy is still unclear, but extended use of rituximab as maintenance agent has shown long-term remission rates, thus limiting the damage accrued by relapsing disease and thus also reducing the risk of end-stage kidney disease (ESKD). Avacopan has been the first agent with a glomerular filtration rate-sparing effect, likely due to more rapid control of kidney inflammation. Those reaching ESKD should be evaluated for kidney transplantation and the risk of remaining on dialysis must be balanced against the risk of recurrence of disease following transplantation.</p><p><strong>Key messages: </strong>The advent of a magnitude of landmark studies in ANCA-GN has refined diagnostic approaches, implemented tools to predict kidney outcome, and eventually led to the approval of newer therapies with avacopan, the latest addition to the armamentarium. Once ESKD is present, patients should be considered for kidney transplantation as remaining on dialysis portends poor overall prognosis.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"26-47"},"PeriodicalIF":0.0,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11842095/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143484968","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"RESOLVE: Recurrence Posttransplant Observational Study in Focal Segmental Glomerulosclerosis and Minimal Change Disease.","authors":"Eloise C Salmon, Ashley E Rahimi, Hailey E Desmond, Nathaniel M Putnam, Elena Martinelli, Elizabeth M Hendren, Susan F Massengill, Priya S Verghese, Simone Sanna-Cherchi, Matthias Kretzler, Abhijit S Naik, Howard Trachtman, Zubin J Modi","doi":"10.1159/000542839","DOIUrl":"10.1159/000542839","url":null,"abstract":"<p><strong>Introduction: </strong>The morbidity of recurrent focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) after transplant is well recognized. Additional collaborative research is necessary to advance understanding of recurrence epidemiology, mechanisms, interventions, and outcomes, particularly in children.</p><p><strong>Methods: </strong>RESOLVE is a multicenter, observational cohort study examining the posttransplant course of patients with FSGS and MCD across the lifespan. Multiple enrollment options will facilitate both retrospective and prospective collection of biospecimens, self-report items, and electronic health record data across pediatric and adult participants. The study offers a unique mobile health option for participants to enroll and engage with the study remotely. Logistic regression using a log link function will evaluate recurrence risk within 3 months of transplant based on clinical characteristics and assess the impact of social determinants of health on time to graft failure, following adjustment. Cox proportional hazards models with primary outcome of graft failure with competing risk of death will evaluate the impact of recurrence therapy and access to preventative versus reactive recurrence therapy. Independent logistic regression will evaluate the impact of recurrence therapy and endophenotypes on proteinuric outcomes.</p><p><strong>Conclusion: </strong>Multiple enrollment approaches and tailored site participation are needed while studying recurrent FSGS (rFSGS) due to its rarity and phenotypic variability. RESOLVE provides a framework for international collaboration to unravel the course of rFSGS through a biospecimen and data repository. It also explores the potential for mobile health tools to enhance recruitment of participants and to promote cooperation among researchers to advance understanding of recurrence mechanisms and treatments.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"60-67"},"PeriodicalIF":0.0,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11842035/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143484638","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Double Trouble in the Basement! A Case of PLA2R-Associated Membranous Nephropathy with Atypical Anti-Glomerular Basement Membrane Disease.","authors":"Renuka Tolani, Steffi Sathiyaraj, David Lyu, Luan Truong, Theresa Thurston, Ziad M El-Zaatari, Ali R Khan, Angelina Edwards, Shane A Bobart","doi":"10.1159/000542859","DOIUrl":"10.1159/000542859","url":null,"abstract":"<p><strong>Introduction: </strong>Concurrent atypical anti-glomerular basement membrane (anti-GBM) disease with membranous nephropathy is a rare occurrence. Compared to typical anti-GBM disease, atypical anti-GBM disease is often seronegative, with a mild disease presentation and course. We present a case of concomitant of PLA2R-associated membranous nephropathy and atypical anti-GBM disease in a patient with cholangiocarcinoma.</p><p><strong>Case presentation: </strong>A 66-year-old male with type 2 diabetes, hypertension, hyperlipidemia, hepatitis C, cirrhosis, and cholangiocarcinoma presented with nephrotic range proteinuria and worsening bilateral lower extremity edema. Urine studies showed 3+ protein and 13 red blood cells per high power field and 24-h urine protein of 14 g (nephrotic range). Serum albumin was 2.1 g/dL and serum creatinine was 0.8 mg/dL (nephrotic syndrome). Serological work-up was negative for antinuclear antibody, anti-double stranded DNA antibody, anti-PLA2R, anti-neutrophil cytoplasmic antibody, rheumatoid factor, and anti-GBM. Complement (C3 and C4) levels were normal and no monoclonal gammopathy was detected. A kidney biopsy showed membranous nephropathy with typical light microscopic, immunofluorescence, and electron microscopic findings. In addition, there was atypical anti-GBM disease characterized by a non-circumferential cellular crescent in 1 out of 12 glomeruli 2-3+ linear staining for IgG along GBM. There was no tubular basement membrane staining for IgG and albumin staining was negative. The glomeruli demonstrated strong staining for PLA2R but were negative for THSD7A and NELL-1. The patient received rituximab infusion, dapagliflozin, and lisinopril, resulting in remission of proteinuria. Despite intense chemotherapy with cisplatin, gemcitabine, and immunotherapy, the patient's cholangiocarcinoma progressed, and he transitioned to hospice care.</p><p><strong>Conclusion: </strong>For our patient, rituximab resulted in remission of proteinuria. The lack of temporal association with the malignancy is consistent with the biopsy findings of PLA2R-associated membranous nephropathy. While there is not an established guideline for atypical anti-GBM disease, our case demonstrates the utility of rituximab for the management of concurrent atypical anti-GBM disease with membranous nephropathy.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"13-18"},"PeriodicalIF":0.0,"publicationDate":"2024-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11842034/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143484272","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Emerging Role of SH3BP2 as Regulator of Immune and Nonimmune Cells in Nephrotic Syndrome.","authors":"Tarak Srivastava, Mukut Sharma","doi":"10.1159/000542703","DOIUrl":"10.1159/000542703","url":null,"abstract":"<p><strong>Background: </strong>Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are major forms of nephrotic syndrome that remain difficult to treat. MCD and FSGS have distinct but also overlapping clinical, histological, metabolic, and molecular features. Effective use of immunosuppressive drugs, activated immune cells, altered cytokine profiles, and upregulated signaling pathways suggest a link between immune dysfunction and nephrotic syndrome, but the exact mechanism of immunopathogenesis is unclear. Immune dysfunction is an area of ongoing research for identifying novel molecular targets for treating nephrotic syndrome. However, the available animal models do not directly address the role of immune dysfunction in nephrotic syndrome.</p><p><strong>Summary: </strong>Genetic analysis indicates that heterogeneous genes related to the podocyte-specific proteins may indirectly cause damage to filtration barrier and influence the onset and progression of nephrotic syndrome. SH3BP2 protein regulates several pathways through its role as a scaffold for many signaling mediators and enzymes. SH3BP2 is expressed in immune as well as in nonimmune cells including podocytes. The role of SH3BP2 is discussed in the context of cells and molecules of adaptive and innate immune systems. Available information on the importance of SH3BP2 in diseases other than nephrotic syndrome and its role in the immunopathogenesis of human nephrotic syndrome are summarized. We outline the key features of a transgenic mouse strain with a gain-in-function mutation (<i>Sh3bp2</i> ^<i>KI/KI</i> ) as a potential model to study immunopathogenesis of nephrotic syndrome.</p><p><strong>Key messages: </strong>Non-receptor, non-catalytic proteins such as SH3BP2 are a novel group of proteins that regulate the innate and adaptive immune responses in nephrotic syndrome. New evidence suggests a critical role of SH3BP2 in immunopathogenesis of nephrotic syndrome. Our recent results demonstrate that transgenic mice (<i>Sh3bp2</i> ^<i>KI/KI</i> ) with a gain-in-function mutation will likely be a unique model to study immunopathogenesis of nephrotic syndrome.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"1-12"},"PeriodicalIF":0.0,"publicationDate":"2024-11-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11842026/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143484273","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}