Targeting WIP1 phosphatase promotes partial remission in experimental collapsing glomerulopathy
Collapsing focal segmental glomerulosclerosis (FSGS), also known as collapsing glomerulopathy (CG), is the most aggressive variant of FSGS and is characterized by a rapid progression to kidney failure. Understanding CG pathogenesis represents a key step for the development of targeted therapies. Previous work implicated the telomerase protein component TERT in CG pathogenesis, as transgenic TERT expression in adult mice resulted in a CG resembling that seen in human primary CG and HIV-associated nephropathy (HIVAN). (Source: Kidney International)
Source: Kidney International - February 27, 2024 Category: Urology & Nephrology Authors: Lou C. Duret, Tynhinane Hamidouche, Nicholas J. Steers, Catherine Pons, Nicolas Soubeiran, Delphine Buret, Eric Gilson, Ali G. Gharavi, Vivette D. D ’Agati, Marina Shkreli Tags: basic research Source Type: research
Paraspeckle protein NONO attenuates vascular calcification by inhibiting bone morphogenetic protein 2 transcription.
Vascular calcification is a pathological process commonly associated with atherosclerosis, chronic kidney disease, and diabetes. Paraspeckle protein NONO is a multifunctional RNA/DNA binding protein involved in many nuclear biological processes but its role in vascular calcification remains unclear. Here, we observed that NONO expression was decreased in calcified arteries of mice and patients with CKD. We generated smooth muscle-specific NONO-knockout mice and established three different mouse models of vascular calcification by means of 5/6 nephrectomy, adenine diet to induce chronic kidney failure, or vitamin D injectio...
Source: Kidney International - February 26, 2024 Category: Urology & Nephrology Authors: Yue Lu, Linlin Meng, Ruiqing Ren, Xinlu Wang, Wenhai Sui, Fei Xue, Lin Xie, Ang Chen, Yuxia Zhao, Jianmin Yang, Wencheng Zhang, Xiao Yu, Bo Xi, Feng Xu, Meng Zhang, Yun Zhang, Cheng Zhang Tags: basic research Source Type: research
A Case-Based Narrative Review of Pregnancy-Associated Atypical Hemolytic Uremic Syndrome/Complement-Mediated Thrombotic Microangiopathy
Atypical hemolytic uremic syndrome (aHUS) is a complement-mediated thrombotic microangiopathy (TMA), caused by uncontrolled activation of the alternative complement pathway in the setting of autoantibodies to or rare pathogenic genetic variants in complement proteins. Pregnancy may serve as a trigger and unmask aHUS/complement-mediated TMA [aHUS/CM-TMA] which has severe, life-threatening consequences. It can be difficult to diagnose aHUS/CM-TMA in pregnancy due to overlapping clinical features with other TMA syndromes including hypertensive disorders of pregnancy. (Source: Kidney International)
Source: Kidney International - February 24, 2024 Category: Urology & Nephrology Authors: Michael Che, Sarah M. Moran, Richard J. Smith, Kevin Y.M. Ren, Graeme N. Smith, M. Khaled Shamseddin, Carmen Avila-Casado, Jocelyn S. Garland Tags: review Source Type: research
Quantitative assessment of glomerular basement membrane collagen IV α chains in paraffin sections from patients with focal segmental glomerulosclerosis and Alport gene variants.
Focal segmental glomerulosclerosis (FSGS) lesions have been linked to variants in COL4A3/A4/A5 genes, which are also mutated in Alport syndrome. Although it could be useful for diagnosis, quantitative evaluation of glomerular basement membrane (GBM) type IV collagen (colIV) networks is not widely used to assess these patients. To do so, we developed immunofluorescence imaging for collagen α5(IV) and α1/2(IV) on kidney paraffin sections with Airyscan confocal microscopy that clearly distinguishes GBM collagen α 3 α 4 α5(IV) and α 1 α 1 α2(IV) as two distinct layers, allowing quantitative assessment of both colIV net...
Source: Kidney International - February 22, 2024 Category: Urology & Nephrology Authors: Pongpratch Puapatanakul, Suramath Isaranuwatchai, Ankanee Chanakul, Jerasit Surintrspanont, Kroonpong Iampenkhae, Talerngsak Kanjanabuch, Kanya Suphapeetiporn, Vivek Charu, Hani Y. Suleiman, Kearkiat Praditpornsilpa, Jeffrey H. Miner Tags: technical notes Source Type: research
KDIGO 2024 Clinical Practice Guideline for the Management of Antineutrophil Cytoplasmic Antibody (ANCA) –Associated Vasculitis
This article is published as part of a supplement sponsored by Kidney Disease: Improving Global Outcomes (KDIGO). The opinions or views expressed in this supplement are those of the authors and do not necessarily reflect the opinions or recommendations of the International Society of Nephrology or Elsevier. Dosages, indications, and methods of use for products that are referred to in the supplement by the authors may reflect their clinical experience or may be derived from the professional literature or other clinical sources. (Source: Kidney International)
Source: Kidney International - February 20, 2024 Category: Urology & Nephrology Authors: Kidney Disease: Improving Global Outcomes (KDIGO) ANCA Vasculitis Work Group Tags: Practice Guideline Source Type: research
Editorial Board
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Source: Kidney International - February 20, 2024 Category: Urology & Nephrology Source Type: research
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Source: Kidney International - February 20, 2024 Category: Urology & Nephrology Source Type: research
Title Page
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Source: Kidney International - February 20, 2024 Category: Urology & Nephrology Source Type: research
Table of Contents
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Source: Kidney International - February 20, 2024 Category: Urology & Nephrology Source Type: research
The role of desmoglein-2 in kidney disease
Desmosomes are multi-protein cell-cell adhesion structures supporting cell stability and mechanical stress resilience of tissues; best described in skin and heart. The kidney is exposed to various mechanical stimuli and stress, yet little is known about kidney desmosomes. In healthy kidneys, we found desmosomal proteins located at the apical-junctional complex in tubular epithelial cells. In four different animal models and patient biopsies with various kidney diseases, desmosomal components were significantly upregulated and partly miss-localized outside of the apical-junctional complexes along the whole lateral tubular e...
Source: Kidney International - February 20, 2024 Category: Urology & Nephrology Authors: Tong Xu, Lea Herkens, Ting Jia, Barbara M. Klinkhammer, Sebastian Kant, Claudia A. Krusche, Eva M. Buhl, Sikander Hayat, J ürgen Floege, Pavel Strnad, Rafael Kramann, Sonja Djudjaj, Peter Boor Tags: basic research Source Type: research
Disrupting circadian control of autophagy induces podocyte injury and proteinuria
The circadian clock influences a wide range of biological process and controls numerous aspects of physiology to adapt to the daily environmental changes caused by Earth ’s rotation. The kidney clock plays an important role in maintaining tubular function, but its effect on podocytes remains unclear. Here, we found that podocytes expressed CLOCK proteins, and that 2666 glomerular gene transcripts (13.4%), including autophagy related genes, had 24-hour circadian rh ythms. Deletion of Clock in podocytes resulted in 1666 gene transcripts with the loss of circadian rhythm including autophagy genes. (Source: Kidney International)
Source: Kidney International - February 20, 2024 Category: Urology & Nephrology Authors: Lulu Wang, Han Tian, Haiyan Wang, Xiaoming Mao, Jing Luo, Qingyun He, Ping Wen, Hongdi Cao, Li Fang, Yang Zhou, Junwei Yang, Lei Jiang Tags: basic research Source Type: research
Reassuring pregnancy outcomes in women with mild COL4A3-5 –related disease (Alport Syndrome) and genetic type of disease can aid personalized counseling
Individualized pre-pregnancy counseling and antenatal care for women with chronic kidney disease (CKD) require disease-specific data. Here, we investigated pregnancy outcomes and long-term kidney function in women with COL4A3-5 related disease (Alport Syndrome, (AS)) in a large multicenter cohort. The ALPART-network (mAternaL and fetal PregnAncy outcomes of women with AlpoRT syndrome), an international collaboration of 17 centers, retrospectively investigated COL4A3-5 related disease pregnancies after the 20th week. (Source: Kidney International)
Source: Kidney International - February 18, 2024 Category: Urology & Nephrology Authors: Margriet E. Gosselink, Rozemarijn Snoek, Agne Cerkauskaite-Kerpauskiene, Sophie P.J. van Bakel, Renee Vollenberg, Henk Groen, Rimante Cerkauskiene, Marius Miglinas, Rossella Attini, K álmán Tory, Kathleen Claes, Kristel van Calsteren, Aude Servais, Marg Tags: clinical investigation Source Type: research
Reassuring pregnancy outcomes in women with mild COL4A3-5 related disease (Alport Syndrome) as the genetic type of disease can aid personalized counseling.
Individualized pre-pregnancy counseling and antenatal care for women with chronic kidney disease (CKD) require disease-specific data. Here, we investigated pregnancy outcomes and long-term kidney function in women with COL4A3-5 related disease (Alport Syndrome, (AS)) in a large multicenter cohort. The ALPART-network (mAternaL and fetal PregnAncy outcomes of women with AlpoRT syndrome), an international collaboration of 17 centers, retrospectively investigated COL4A3-5 related disease pregnancies after the 20th week. (Source: Kidney International)
Source: Kidney International - February 18, 2024 Category: Urology & Nephrology Authors: Margriet E. Gosselink, Rozemarijn Snoek, Agne Cerkauskaite-Kerpauskiene, Sophie P.J. van Bakel, Renee Vollenberg, Henk Groen, Rimante Cerkauskiene, Marius Miglinas, Rossella Attini, K álmán Tory, Kathleen Claes, Kristel van Calsteren, Aude Servais, Marg Tags: clinical investigation Source Type: research
A Neanderthal haplotype introgressed into the human genome confers protection against membranous nephropathy
Class 2 HLA and PLA2R1 alleles are exceptionally strong genetic risk factors for membranous nephropathy (MN), leading, through an unknown mechanism, to a targeted autoimmune response. Introgressed archaic haplotypes (introduced from an archaic human genome into the modern human genome) might influence phenotypes through gene dysregulation. Here, we investigated the genomic region surrounding the PLA2R1 gene. We reconstructed the phylogeny of Neanderthal and modern haplotypes in this region and calculated the probability of the observed clustering being the result of introgression or common descent. (Source: Kidney International)
Source: Kidney International - February 14, 2024 Category: Urology & Nephrology Authors: Catalin D. Voinescu, Monika Mozere, Giulio Genovese, Mallory L. Downie, Sanjana Gupta, Daniel P. Gale, Detlef Bockenhauer, Robert Kleta, Mauricio Arcos-Burgos, Horia C. Stanescu Tags: Basic Research Source Type: research
An update on clinical presentation and responses to therapy of patients with hereditary hypophosphatemic rickets with hypercalciuria (HHRH).
Pathogenic variants in solute carrier family 34, member 3 (SLC34A3), the gene encoding the sodium-dependent phosphate cotransporter 2c (NPT2c), cause hereditary hypophosphatemic rickets with hypercalciuria (HHRH). Here, we report a pooled analysis of clinical and laboratory records of 304 individuals from 145 kindreds, including 20 previously unreported HHRH kindreds, in which two novel SLC34A3 pathogenic variants were identified. Compound heterozygous/homozygous carriers show above 90% penetrance for kidney and bone phenotypes. (Source: Kidney International)
Source: Kidney International - February 14, 2024 Category: Urology & Nephrology Authors: Zewu Zhu, Bryan Bo-Ran Ho, Alyssa Chen, James Amrhein, Andreea Apetrei, Thomas Oliver Carpenter, Marise Lazaretti-Castro, Juan Manuel Colazo, Kathryn McCrystal Dahir, Michaela Ge ßner, Evgenia Gurevich, Cathrine Alsaker Heier, Jill Hickman Simmons, Tracy Tags: clinical investigation Source Type: research
