Glomerular diseases: genetic causes and future therapeutics.

Nature Reviews. Nephrology
Chih-Kang Chiang, Reiko Inagi

Abstract

The glomerulus consists of capillary tufts, a mesangial cell component and the Bowman capsule. The glomerular filtration barrier is composed of glomerular endothelial cells, a basement membrane, and podocytes. Particular components of the slit diaphragm and the glomerular basement membrane strictly orchestrate the integrity of the glomerular filtration barrier. The basement membrane is made of a highly crosslinked macromolecular meshwork of type IV collagen, proteoglycans, and laminin. Genetic forms of glomerular disease are predominantly caused by genetic defects in these molecular structures or in factors that regulate the glomerular filtration barrier. In addition, abnormal IgA1 glycosylation can increase susceptibility to IgA nephropathy. Dysregulation of the complement system or of platelet activation can lead to the development of endocapillary lesions, which manifest as thrombotic microangiopathy. Glomerular dysfunction is also encountered in several genetic metabolic and mitochondrial disorders. Discoveries of mutations in a range of genes have provided new insights into the mechanisms of glomerular disease. In this Review, we summarize recent progress in the genetics and therapeutics of a number of glomerular diseases.

References

Feb 1, 1990·Proceedings of the National Academy of Sciences of the United States of America·S L HostikkaK Tryggvason
Feb 1, 1990·Proceedings of the National Academy of Sciences of the United States of America·C G SaezL A Leinwand
Feb 1, 1989·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·T SaitoH Sakaguchi
Oct 1, 1988·American Journal of Medical Genetics·R Gershoni-BaruchC Lichtig
Mar 1, 1972·The American Journal of Medicine·C J EpsteinA R Ablin
Feb 13, 1981·JAMA : the Journal of the American Medical Association·L CarrerasJ Alsina
Aug 1, 1994·Matrix Biology : Journal of the International Society for Matrix Biology·R Timpl, J C Brown
Feb 1, 1994·Annals of Internal Medicine·G M TalenteJ I Wolfsdorf
Jul 1, 1997·Journal of the American Society of Nephrology : JASN·J J JansenH H Lemkes
Apr 29, 1998·The Journal of Cell Biology·K HondaS Hirohashi
Apr 29, 1998·Kidney International·P WarwickerJ A Goodship
Jul 17, 1998·Developmental Genetics·H ChenR L Johnson
Feb 23, 1999·Kidney International·L CallísJ Nieto
Jul 8, 1999·Proceedings of the National Academy of Sciences of the United States of America·V RuotsalainenK Tryggvason
Oct 3, 1999·Kidney International·L B HolzmanD R Abrahamson
May 20, 2000·DNA Research : an International Journal for Rapid Publication of Reports on Genes and Genomes·T NagaseO Ohara
Jun 30, 2000·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·N KambhamV D D'Agati
Oct 24, 2000·Kidney International·F T van der LoopH J Smeets
Apr 24, 2001·Human Mutation·O BeltchevaK Tryggvason
Jun 21, 2001·JAMA : the Journal of the American Medical Association·R SchiffmannR O Brady
Jul 7, 2001·The New England Journal of Medicine·C M EngUNKNOWN International Collaborative Fabry Disease Study Group

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Citations

Nov 28, 2012·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Helen Liapis, Joseph P Gaut
Jul 16, 2011·The New England Journal of Medicine·Caterina MeleUNKNOWN PodoNet Consortium
Apr 23, 2014·Nature Reviews. Nephrology·Reiko InagiMasaomi Nangaku
Oct 2, 2014·Proceedings of the National Academy of Sciences of the United States of America·Minna D BalbasCharles L Sawyers
Jul 7, 2012·Cellular and Molecular Life Sciences : CMLS·Andreas BauwensJohannes Müthing
Mar 14, 2014·Kidney International·William G Couser, Richard J Johnson
Aug 16, 2011·Seminars in Nephrology·Alain Meyrier
Jul 26, 2011·Advances in Chronic Kidney Disease·Tino D Piscione, Christoph Licht
Aug 3, 2011·Journal of Signal Transduction·Andrey V Cybulsky, Chris R J Kennedy
Jun 13, 2014·Genesis : the Journal of Genetics and Development·Paul T Kroeger, Rebecca A Wingert
Sep 11, 2012·Seminars in Nephrology·Wilhelm Kriz, Karlhans Endlich
Apr 3, 2018·BMC Biotechnology·Tomoe KobayashiMakoto Matsuyama
Dec 1, 2017·American Journal of Physiology. Renal Physiology·Andrey V CybulskyElena Torban
Jun 7, 2018·American Journal of Physiology. Renal Physiology·Albert YeeAndrey V Cybulsky
Feb 27, 2016·F1000Research·Jochen Reiser, Mehmet M Altintas
Jan 28, 2012·Journal of the American Society of Nephrology : JASN·William G Couser
Nov 27, 2014·Journal of the American Society of Nephrology : JASN·Shuta MotonishiReiko Inagi
Apr 21, 2017·Journal of the American Society of Nephrology : JASN·Ruchika SharmaBryce A Kerlin
Jun 13, 2012·Nature Reviews. Nephrology·Larry A GreenbaumWilliam E Smoyer
Dec 18, 2012·American Journal of Physiology. Cell Physiology·Chih-Kang ChiangReiko Inagi
Jan 1, 2018·Medizinische Genetik : Mitteilungsblatt des Berufsverbandes Medizinische Genetik e.V·Wouter J C FeitzChristoph Licht
May 27, 2016·The Journal of Biological Chemistry·Hanan ElimamAndrey V Cybulsky
Apr 22, 2017·Molecular Biology of the Cell·Daniel Robert KaufmanAndrey V Cybulsky
Feb 24, 2017·Frontiers in Physiology·Hiroshi Maekawa, Reiko Inagi
May 22, 2013·Journal of the American Animal Hospital Association·Marco PierantozziAndrea Zatelli
Feb 6, 2021·Journal of Clinical Medicine·Shipra Agrawal On Behalf Of The Pediatric Nephrology Research Consortium Pnrc

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