Structural Basis of Highly Specific Interaction between Nephrin and MAGI1 in Slit Diaphragm Assembly and Signaling
Conclusions MAGI1 interacts with nephrin-PBM with exquisite specificity. A newly developed, potent inhibitory peptide that blocks this interaction may be useful for future functional investigations in vivo. Our findings also provide possible explanations for the diseases caused by NPHS1 mutations. (Source: Journal of the American Society of Nephrology : JASN)
Source: Journal of the American Society of Nephrology : JASN - August 31, 2018 Category: Urology & Nephrology Authors: Weng, Z., Shang, Y., Ji, Z., Ye, F., Lin, L., Zhang, R., Zhu, J. Tags: Basic Research Source Type: research

Whole-Exome Sequencing Identifies Causative Mutations in Families with Congenital Anomalies of the Kidney and Urinary Tract
Conclusions We identified monogenic mutations in a known human CAKUT gene or CAKUT phenocopy gene as the cause of disease in 14% of the CAKUT families in this study. Whole-exome sequencing provides an etiologic diagnosis in a high fraction of patients with CAKUT and will provide a new basis for the mechanistic understanding of CAKUT. (Source: Journal of the American Society of Nephrology : JASN)
Source: Journal of the American Society of Nephrology : JASN - August 31, 2018 Category: Urology & Nephrology Authors: van der Ven, A. T., Connaughton, D. M., Ityel, H., Mann, N., Nakayama, M., Chen, J., Vivante, A., Hwang, D.-y., Schulz, J., Braun, D. A., Schmidt, J. M., Schapiro, D., Schneider, R., Warejko, J. K., Daga, A., Majmundar, A. J., Tan, W., Jobst-Schwan, T., H Tags: Basic Research Source Type: research

ABCC6 Deficiency Promotes Development of Randall Plaque
Conclusions The frequency of kidney stones and probably, Randall plaque is extremely high in patients with PXE, and Abcc6–/– mice provide a new and useful model in which to study Randall plaque formation. Our findings also suggest that pyrophosphate administration should be evaluated for the prevention of Randall plaque and kidney stones. (Source: Journal of the American Society of Nephrology : JASN)
Source: Journal of the American Society of Nephrology : JASN - August 31, 2018 Category: Urology & Nephrology Authors: Letavernier, E., Kauffenstein, G., Huguet, L., Navasiolava, N., Bouderlique, E., Tang, E., Delaitre, L., Bazin, D., de Frutos, M., Gay, C., Perez, J., Verpont, M.-C., Haymann, J.-P., Pomozi, V., Zoll, J., Le Saux, O., Daudon, M., Leftheriotis, G., Martin, Tags: Basic Research Source Type: research

Phosphorylation of Acetyl-CoA Carboxylase by AMPK Reduces Renal Fibrosis and Is Essential for the Anti-Fibrotic Effect of Metformin
Conclusions These data indicate that reduced phosphorylation of ACC after renal injury contributes to the development of TIF, and that phosphorylation of ACC is required for metformin’s antifibrotic action in the kidney. (Source: Journal of the American Society of Nephrology : JASN)
Source: Journal of the American Society of Nephrology : JASN - August 31, 2018 Category: Urology & Nephrology Authors: Lee, M., Katerelos, M., Gleich, K., Galic, S., Kemp, B. E., Mount, P. F., Power, D. A. Tags: Basic Research Source Type: research

Interactions between Macrophages and Cyst-Lining Epithelial Cells Promote Kidney Cyst Growth in Pkd1-Deficient Mice
Conclusions Interactions between macrophages and CLECs promote cyst growth. ARG1 is a key molecule involved in this process and is a potential therapeutic target to help delay ADPKD progression. (Source: Journal of the American Society of Nephrology : JASN)
Source: Journal of the American Society of Nephrology : JASN - August 31, 2018 Category: Urology & Nephrology Authors: Yang, Y., Chen, M., Zhou, J., Lv, J., Song, S., Fu, L., Chen, J., Yang, M., Mei, C. Tags: Basic Research Source Type: research

Biallelic Expression of Mucin-1 in Autosomal Dominant Tubulointerstitial Kidney Disease: Implications for Nongenetic Disease Recognition
Conclusions Diagnosing ADTKD-MUC1 by molecular genetics is possible, but it is technically demanding and labor intensive. However, immunohistochemistry on kidney biopsy specimens is feasible for nongenetic diagnosis of ADTKD-MUC1 and therefore, a valid method to select families for further diagnostics. Our data are compatible with the hypothesis that specific molecular effects of MUC1-fs underlie the pathogenesis of this disease. (Source: Journal of the American Society of Nephrology : JASN)
Source: Journal of the American Society of Nephrology : JASN - August 31, 2018 Category: Urology & Nephrology Authors: Knaup, K. X., Hackenbeck, T., Popp, B., Stoeckert, J., Wenzel, A., Büttner-Herold, M., Pfister, F., Schueler, M., Seven, D., May, A. M., Halbritter, J., Gröne, H.-J., Reis, A., Beck, B. B., Amann, K., Ekici, A. B., Wiesener, M. S. Tags: Basic Research Source Type: research

Efficient Gene Transfer to Kidney Mesenchymal Cells Using a Synthetic Adeno-Associated Viral Vector
Conclusions These studies establish a novel method for inducible knockout of floxed genes in mouse mesangium, pericytes, and perivascular fibroblasts and are the foundation for future gene therapy approaches to treat kidney fibrosis. (Source: Journal of the American Society of Nephrology : JASN)
Source: Journal of the American Society of Nephrology : JASN - August 31, 2018 Category: Urology & Nephrology Authors: Ikeda, Y., Sun, Z., Ru, X., Vandenberghe, L. H., Humphreys, B. D. Tags: Basic Research Source Type: research

Pretransplant C3d-Fixing Donor-Specific Anti-HLA Antibodies Are Not Associated with Increased Risk for Kidney Graft Failure
Conclusions The C3d-fixing ability of pretransplant DSA is not associated with increased risk for graft failure. (Source: Journal of the American Society of Nephrology : JASN)
Source: Journal of the American Society of Nephrology : JASN - August 31, 2018 Category: Urology & Nephrology Authors: Kamburova, E. G., Wisse, B. W., Joosten, I., Allebes, W. A., van der Meer, A., Hilbrands, L. B., Baas, M. C., Spierings, E., Hack, C. E., van Reekum, F. E., van Zuilen, A. D., Verhaar, M. C., Bots, M. L., Drop, A. C. A. D., Plaisier, L., Seelen, M. A. J., Tags: Rapid Communication Source Type: research

Effects of Enzyme Replacement Therapy and Antidrug Antibodies in Patients with Fabry Disease
(Source: Journal of the American Society of Nephrology : JASN)
Source: Journal of the American Society of Nephrology : JASN - August 31, 2018 Category: Urology & Nephrology Authors: Lenders, M., Brand, E. Tags: Review Source Type: research

What Is the Glomerular Ultrafiltration Barrier?
(Source: Journal of the American Society of Nephrology : JASN)
Source: Journal of the American Society of Nephrology : JASN - August 31, 2018 Category: Urology & Nephrology Authors: Fissell, W. H., Miner, J. H. Tags: Perspective Source Type: research

A New Pediatric AKI Definition: Implications of Trying to Build the Perfect Mousetrap
(Source: Journal of the American Society of Nephrology : JASN)
Source: Journal of the American Society of Nephrology : JASN - August 31, 2018 Category: Urology & Nephrology Authors: Goldstein, S. L. Tags: Editorials Source Type: research

MUC1 Makes Me Miserable
(Source: Journal of the American Society of Nephrology : JASN)
Source: Journal of the American Society of Nephrology : JASN - August 31, 2018 Category: Urology & Nephrology Authors: Gale, D. P., Kleta, R. Tags: Editorials Source Type: research

This Month's Highlights
(Source: Journal of the American Society of Nephrology : JASN)
Source: Journal of the American Society of Nephrology : JASN - August 31, 2018 Category: Urology & Nephrology Tags: This Month ' s Highlights Source Type: research

Erratum
(Source: Journal of the American Society of Nephrology : JASN)
Source: Journal of the American Society of Nephrology : JASN - July 31, 2018 Category: Urology & Nephrology Tags: Errata Source Type: research