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Advances in our understanding of genetic kidney disease using kidney organoids

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Abstract

A significant proportion of kidney disease presenting in childhood is likely genetic in origin with a growing number of genes implicated in its development. However, many children may have changes in previously undescribed or unrecognised genes. The recent development of methods for generating human kidney organoids from human pluripotent stem cells has the potential to substantially change the rate of diagnosis and the development of new treatments for some forms of genetic kidney disease. In this review, we discuss how accurately a kidney organoid models the human kidney, identifying the strengths and weaknesses of these potentially patient-derived models of renal disease.

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Acknowledgements

We thank Tom Forbes, Lorna Hale, Sara Howden, Belinda Phipson, Alexander Combes, Alicia Oshlack, Andrew Mallett, Cathelijne van den Berg, Ton Rabelink and others whose work we have referred to. We also acknowledge Dr. Chirag Patel, Dr. Andrew Mallett and the Kidgen Collaborative (www.kidgen.org.au) for the kidney gene list represented in Table 1 and in use within the Victorian Clinical Genetics Service (www.vcgs.org.au). MHL is an NHMRC Senior Principal Research Fellow with the National Health and Medical Research Council (GNT1136085).

Funding

This work was supported by the NHMRC (GNT1098654) and the Royal Children’s Hospital Foundation RenGeniPS program.

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Authors and Affiliations

  1. Murdoch Children’s Research Institute, Flemington Rd., Parkville, VIC, Australia

    Melissa H. Little & Catherine Quinlan

  2. Department of Anatomy and Neuroscience, The University of Melbourne, Melbourne, VIC, Australia

    Melissa H. Little

  3. Department of Paediatrics, The University of Melbourne, Melbourne, VIC, Australia

    Melissa H. Little & Catherine Quinlan

  4. Department of Nephrology, Royal Children’s Hospital, Flemington Rd., Parkville, VIC, Australia

    Catherine Quinlan

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  1. Melissa H. Little
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Little, M.H., Quinlan, C. Advances in our understanding of genetic kidney disease using kidney organoids. Pediatr Nephrol 35, 915–926 (2020). https://doi.org/10.1007/s00467-019-04259-x

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