Literature DB >> 33009821

Bedside to bench Alport syndrome research: are human urine-derived podocytes the answer?.

Jin-Ju Kim1, Alessia Fornoni1.   

Abstract

In a recent issue of The Journal of Pathology, Iampietro et al isolated and characterized several clones of urine-derived podocytes from three patients with Alport syndrome (AS) and proteinuria and one age-matched non-proteinuric control. They reported differential expression of genes involved in cell motility, adhesion, survival, and angiogenesis. The authors found AS podocytes to be less motile and to have significantly higher permeability to albumin compared to control podocytes, highlighting that AS podocytes may retain their phenotype even when losing contact with the glomerular basement membrane. The establishment of urine-derived podocyte cell lines from patients with different genetic forms of AS may represent a valuable and minimally invasive tool to investigate the cellular mechanisms contributing to kidney disease progression in AS and may allow for the establishment of patient-specific drug screening opportunities.
© 2020 The Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd. © 2020 The Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

Entities:  

Keywords:  Alport syndrome; collagen IV mutation; glomerular basement membrane; glomerular filtration barrier; urinary podocytes

Mesh:

Year:  2020        PMID: 33009821      PMCID: PMC8142897          DOI: 10.1002/path.5564

Source DB:  PubMed          Journal:  J Pathol        ISSN: 0022-3417            Impact factor:   7.996


  17 in total

Review 1.  Alport's syndrome, Goodpasture's syndrome, and type IV collagen.

Authors:  Billy G Hudson; Karl Tryggvason; Munirathinam Sundaramoorthy; Eric G Neilson
Journal:  N Engl J Med       Date:  2003-06-19       Impact factor: 91.245

2.  Cellular origins of type IV collagen networks in developing glomeruli.

Authors:  Dale R Abrahamson; Billy G Hudson; Larysa Stroganova; Dorin-Bogdan Borza; Patricia L St John
Journal:  J Am Soc Nephrol       Date:  2009-05-07       Impact factor: 10.121

3.  Glomerular basement membrane. Identification of a novel disulfide-cross-linked network of alpha3, alpha4, and alpha5 chains of type IV collagen and its implications for the pathogenesis of Alport syndrome.

Authors:  S Gunwar; F Ballester; M E Noelken; Y Sado; Y Ninomiya; B G Hudson
Journal:  J Biol Chem       Date:  1998-04-10       Impact factor: 5.157

4.  Diagnostic Utility of Exome Sequencing for Kidney Disease. Reply.

Authors:  Emily Groopman; David Goldstein; Ali Gharavi
Journal:  N Engl J Med       Date:  2019-05-23       Impact factor: 91.245

5.  The R229Q mutation in NPHS2 may predispose to proteinuria in thin-basement-membrane nephropathy.

Authors:  Stephen Tonna; Yan Yan Wang; Diane Wilson; Lin Rigby; Tania Tabone; Richard Cotton; Judy Savige
Journal:  Pediatr Nephrol       Date:  2008-08-26       Impact factor: 3.714

6.  Differential expression of two basement membrane collagen genes, COL4A6 and COL4A5, demonstrated by immunofluorescence staining using peptide-specific monoclonal antibodies.

Authors:  Y Ninomiya; M Kagawa; K Iyama; I Naito; Y Kishiro; J M Seyer; M Sugimoto; T Oohashi; Y Sado
Journal:  J Cell Biol       Date:  1995-09       Impact factor: 10.539

7.  Targeted next-generation sequencing in steroid-resistant nephrotic syndrome: mutations in multiple glomerular genes may influence disease severity.

Authors:  Gemma Bullich; Daniel Trujillano; Sheila Santín; Stephan Ossowski; Santiago Mendizábal; Gloria Fraga; Álvaro Madrid; Gema Ariceta; José Ballarín; Roser Torra; Xavier Estivill; Elisabet Ars
Journal:  Eur J Hum Genet       Date:  2014-11-19       Impact factor: 4.246

8.  Vinculin is required to maintain glomerular barrier integrity.

Authors:  Franziska Lausecker; Xuefei Tian; Kazunori Inoue; Zhen Wang; Christopher E Pedigo; Hossam Hassan; Chang Liu; Margaret Zimmer; Stephanie Jinno; Abby L Huckle; Hellyeh Hamidi; Robert S Ross; Roy Zent; Christoph Ballestrem; Rachel Lennon; Shuta Ishibe
Journal:  Kidney Int       Date:  2017-12-12       Impact factor: 10.612

9.  DNA-Encoded Library-Derived DDR1 Inhibitor Prevents Fibrosis and Renal Function Loss in a Genetic Mouse Model of Alport Syndrome.

Authors:  Hans Richter; Alexander L Satz; Marc Bedoucha; Bernd Buettelmann; Ann C Petersen; Anja Harmeier; Ricardo Hermosilla; Remo Hochstrasser; Dominique Burger; Bernard Gsell; Rodolfo Gasser; Sylwia Huber; Melanie N Hug; Buelent Kocer; Bernd Kuhn; Martin Ritter; Markus G Rudolph; Franziska Weibel; Judith Molina-David; Jin-Ju Kim; Javier Varona Santos; Martine Stihle; Guy J Georges; R Daniel Bonfil; Rafael Fridman; Sabine Uhles; Solange Moll; Christian Faul; Alessia Fornoni; Marco Prunotto
Journal:  ACS Chem Biol       Date:  2018-12-16       Impact factor: 5.100

10.  Molecular and functional characterization of urine-derived podocytes from patients with Alport syndrome.

Authors:  Corinne Iampietro; Linda Bellucci; Fanny O Arcolino; Maddalena Arigoni; Luca Alessandri; Yonathan Gomez; Elli Papadimitriou; Raffaele A Calogero; Enrico Cocchi; Lambertus Van Den Heuvel; Elena Levtchenko; Benedetta Bussolati
Journal:  J Pathol       Date:  2020-09       Impact factor: 7.996

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