ReviewClinical journal of the American Society of Nephrology : CJASN2020
Disease Modeling To Understand the Pathomechanisms of Human Genetic Kidney Disorders.
Review in Clinical journal of the American Society of Nephrology : CJASN, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 13 citations in OpenAlex.
- A High-fat, High-salt Diet Model of MDAKD Impairs Bioenergetic Efficiency for ATP Synthesis.bioRxiv : the preprint server for biology · 2026Article
- Kidney Fibrosis In Vitro and In Vivo Models: Path Toward Physiologically Relevant Humanized Models.Advanced healthcare materials · 2025Review
- Esrrγa regulates nephron and ciliary development by controlling prostaglandin synthesis.Development (Cambridge, England) · 2023Article
- Principles of Zebrafish Nephron Segment Development.Journal of developmental biology · 2023Review
- Article
- Modeling Podocyte Ontogeny and Podocytopathies with the Zebrafish.Journal of developmental biology · 2023Review
- Review
- Article
- Organs-on-chip technology: a tool to tackle genetic kidney diseases.Pediatric nephrology (Berlin, Germany) · 2022Review
- Engineering of thick human functional myocardium via static stretching and electrical stimulation.iScience · 2022Article
- Zebrafish for Personalized Regenerative Medicine; A More Predictive Humanized Model of Endocrine Disease.Frontiers in endocrinology · 2020Review
- A biomimetic tubuloid-on-a-chip for human renal fibrosis research and anti-fibrotic drug development.Journal of tissue engineeringArticle
Corrections and comments
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Authors and funding
2 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The class of human genetic kidney diseases is extremely broad and heterogeneous. Accordingly, the range of associated disease phenotypes is highly variable. Many children and adults affected by inherited kidney disease will progress to ESKD at some point in life. Extensive research has been performed on various different disease models to investigate the underlying causes of genetic kidney disease and to identify disease mechanisms that are amenable to therapy. We review some of the research highlights that, by modeling inherited kidney disease, contributed to a better understanding of the underlying pathomechanisms, leading to the identification of novel genetic causes, new therapeutic targets, and to the development of new treatments. We also discuss how the implementation of more efficient genome-editing techniques and tissue-culture methods for kidney research is providing us with personalized models for a precision-medicine approach that takes into account the specificities of the patient and the underlying disease. We focus on the most common model systems used in kidney research and discuss how, according to their specific features, they can differentially contribute to biomedical research. Unfortunately, no definitive treatment exists for most inherited kidney disorders, warranting further exploitation of the existing disease models, as well as the implementation of novel, complex, human patient-specific models to deliver research breakthroughs.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.