Review
Medicine, General & Internal
Xuefei Tian, Patricia Bunda, Shuta Ishibe
Summary: Endocytosis is a mechanism that internalizes and recycles plasma membrane components and transmembrane receptors via vesicle formation. Podocytes, specialized epithelial cells in the kidney, play a critical role in maintaining the integrity of the glomerular filtration barrier. Dysfunction of podocyte endocytosis is associated with the development and progression of proteinuria in chronic kidney disease. Understanding the mechanism of podocyte endocytosis may provide insights into maintaining normal filtration function and potential therapeutic strategies for proteinuric glomerular diseases.
FRONTIERS IN MEDICINE
(2022)
Article
Urology & Nephrology
Andrew J. B. Watts, Keith H. Keller, Gabriel Lerner, Ivy Rosales, A. Bernard Collins, Miroslav Sekulic, Sushrut S. Waikar, Anil Chandraker, Leonardo V. Riella, Mariam P. Alexander, Jonathan P. Troost, Junbo Chen, Damian Fermin, Jennifer L. Yee, Matthew G. Sampson, Laurence H. H. Beck Jr, Joel M. Henderson, Anna Greka, Helmut G. Rennke, Astrid Weins
Summary: In a subset of patients with minimal change disease, circulating nephrin autoantibodies were detected during active disease but significantly reduced or absent during treatment response. These autoantibodies were correlated with podocyte-associated punctate IgG in renal biopsies. A steroid-dependent patient with childhood minimal change disease developed end stage kidney disease, with high pretransplant circulating nephrin autoantibodies associated with massive post-transplant recurrence of proteinuria.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2022)
Article
Immunology
Qing Ye, Dongjie Wang, Chao Zhou, Hanyan Meng, Huihui Liu, Jianhua Mao
Summary: Idiopathic nephrotic syndrome (INS) is a common renal disease characterized by disruption of the glomerular filtration barrier. In a previous study, podocyte autoantibodies were identified in INS patients, leading to the concept of autoimmune podocytopathy. This study aims to investigate the presence of autoantibodies against vascular endothelial cells in INS patients and assess their clinical significance and pathogenicity. Screening of INS patient sera revealed nine types of autoantibodies against vascular endothelial cells, potentially causing endothelial cell damage. Additionally, 89% of the patients tested positive for at least one autoantibody.
CLINICAL IMMUNOLOGY
(2023)
Article
Urology & Nephrology
Yizhen Chen, Aicheng Yang, Yuansheng Hou, Longhui Liu, Jiehua Lin, Xiaodan Huang, Jundu Li, Xusheng Liu, Fuhua Lu, Qizhan Lin, Haifeng Yang, Shuling Yue, Shujun Jiang, Lixin Wang, Chuan Zou
Summary: Nephrotic syndrome (NS) and nephrotic-range proteinuria (NRP) are uncommon in IgA nephropathy (IgAN) and have distinct clinicopathological characteristics. Podocytes play an important role in both types. NS is associated with more severe lesions and foot process effacement (FPE) compared to NRP. NRP is an independent risk factor for renal progression, while moderate FPE (MFPE) is associated with a better prognosis in NRP patients without obvious chronic renal lesions.
Article
Medicine, General & Internal
Xu He, Tao Sun, Pei Zhang, Zhengkun Xia, Chunlin Gao, Hongqi Ren, Daxi Ji
Summary: Inhibition of HDAC IIa improves podocyte injury and proteinuria, suggesting the potential use of MC1568 in the treatment of nephrotic syndrome.
FRONTIERS IN MEDICINE
(2022)
Review
Health Care Sciences & Services
Mengyuan Ge, Sandra Merscher, Alessia Fornoni
Summary: Although guidelines for managing hyperlipidemia in nephrotic syndrome are largely opinion-based, recent studies suggest that intrarenal lipids play a role in the progression of glomerular diseases. Reducing intracellular lipids may represent a new therapeutic approach to slow the progression of chronic kidney disease.
JOURNAL OF PERSONALIZED MEDICINE
(2021)
Article
Urology & Nephrology
Mahmoud Kallash, Yujie Wang, Abigail Smith, Howard Trachtman, Rasheed Gbadegesin, Carla Nester, Pietro Canetta, Chen Wang, Tracy E. Hunley, C. John Sperati, David Selewski, Isabelle Ayoub, Tarak Srivastava, Amy K. Mottl, Jeffrey Kopp, Brenda Gillespie, Bruce Robinson, Dhruti Chen, Julia Steinke, Katherine Twombley, Kimberly Reidy, Krzysztof Mucha, Larry A. Greenbaum, Brooke Blazius, Margaret Helmuth, Peleg Yonatan, Rulan S. Parekh, Susan Hogan, Virginie Royal, Vivette D'Agati, Aftab Chishti, Ronald Falk, Ali Gharavi, Lawrence Holzman, Jon Klein, William Smoyer, Matthias Kretzler, Debbie Gipson, Jason M. Kidd
Summary: Background: FSGS is a heterogeneous diagnosis with a guarded prognosis. APOL1 gene polymorphisms are associated with developing FSGS and faster progression to kidney failure. Understanding the natural history of FSGS patients with APOL1 risk alleles is important for patient care and intervention studies.
CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2023)
Article
Urology & Nephrology
Alla Mitrofanova, Antonio Fontanella, Matthew Tolerico, Shamroop Mallela, Judith Molina David, Yiqin Zuo, Marcia Boulina, Jin-Ju Kim, Javier Santos, Mengyuan Ge, Alexis Sloan, Wadih Issa, Margaret Gurumani, Jeffrey Pressly, Marie Ito, Matthias Kretzler, Sean Eddy, Robert Nelson, Sandra Merscher, George Burke, Alessia Fornoni
Summary: This study reveals the crucial role of STING activation in the progression of DKD and Alport syndrome, suggesting that targeting STING may provide a therapeutic option for glomerular diseases.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2022)
Article
Pediatrics
Martin Bezdicka, Dana Zemkova, Sylva Skalova, Eva Hovorkova, Miroslav Podhola, Jan Burkert, Jakub Zieg
Summary: Monogenic nephrotic syndrome is associated with a resistance to initial glucocorticoid therapy and causative variants in genes influencing podocyte stability and kidney development. Mutations in the TTC21B gene can lead to proteinuric diseases, including nephrotic syndrome, and clinical features such as glomerular proteinuric disease with tubulointestial involvement and skeletal abnormalities.
FRONTIERS IN PEDIATRICS
(2021)
Review
Urology & Nephrology
George Vasquez-Rios, Marina De Cos, Kirk N. Campbell
Summary: Apolipoprotein L1 (APOL1) high-risk variants are associated with an increased risk for kidney disease. Recent studies have provided mechanistic insights into the development of APOL1-mediated kidney diseases (AMKD) and proposed therapeutic strategies to improve clinical outcomes.
KIDNEY INTERNATIONAL REPORTS
(2023)
Review
Medicine, General & Internal
Shrey Purohit, Federica Piani, Flor A. Ordonez, Carmen de Lucas-Collantes, Colin Bauer, Gabriel Cara-Fuentes
Summary: Minimal change disease (MCD) is the most common type of idiopathic nephrotic syndrome in childhood, characterized by massive proteinuria, edema, and podocyte foot process effacement. Studies suggest an association between MCD and immune dysregulation, possibly mediated by unknown factors released by T cells. The pathogenesis of the disease remains unknown, with recent focus on the role of B cells and endothelial cells in MCD.
FRONTIERS IN MEDICINE
(2021)
Review
Urology & Nephrology
Ponticelli Claudio, Moroni Gabriella
Summary: Nephrotic syndrome in patients with kidney disease can lead to various complications, including progressive kidney dysfunction. Proteinuria can cause the formation of cellular or fibrous crescents, leading to rapidly progressive glomerulonephritis or focal glomerulosclerosis. It can also result in tubular atrophy and interstitial fibrosis. Other complications include hypoalbuminemia, dyslipidemia, hypercoagulable state, and increased risk of infection. Treating these complications can reduce the severity of nephrotic syndrome.
JOURNAL OF NEPHROLOGY
(2023)
Article
Biochemistry & Molecular Biology
Saaya Hatakeyama, Akihiro Tojo, Hiroshi Satonaka, Nami O. Yamada, Takao Senda, Toshihiko Ishimitsu
Summary: In a study on mice lacking APC, it was found that podocyte vesicle transport was suppressed under induction of minimal change nephrotic syndrome, leading to reduced albuminuria compared to wild-type mice. This suggests a crucial role of APC in maintaining podocyte vesicle transport and renal function during nephrotic syndrome development.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Immunology
Qing Ye, Chao Zhou, Dongjie Wang, Haidong Fu, Jingjing Wang, Jianhua Mao
Summary: This study screened and identified pathogenic podocyte autoantibodies in children with INS using two-dimensional electrophoresis and mass spectrometry. It found that at least 66% of INS children have these podocyte autoantibodies, which are closely related to specific clinical manifestations.
CLINICAL IMMUNOLOGY
(2021)
Article
Medical Laboratory Technology
Samuel Doiron, Martine Paquette, Alexis Baass, Guillaume Bollee, Heloise Cardinal, Sophie Bernard
Summary: This study investigated the association between the magnitude of proteinuria and PCSK9 concentrations. The results showed that PCSK9 was significantly elevated in patients with nephrotic syndrome and was associated with a detrimental lipid profile.
CLINICAL BIOCHEMISTRY
(2022)