Abstract
Several animal- and human-derived models are used in autosomal dominant polycystic kidney disease (ADPKD) research to gain insight in the disease mechanism. However, a consistent correlation between animal and human ADPKD models is lacking. Therefore, established human-derived models are relevant to affirm research results and translate findings into a clinical set-up. In this review, we give an extensive overview of the existing human-based cell models. We discuss their source (urine, nephrectomy and stem cell), immortalisation procedures, genetic engineering, kidney segmental origin and characterisation with nephron segment markers. We summarise the most studied pathways and lessons learned from these different ADPKD models. Finally, we issue recommendations for the derivation of human-derived cell lines and for experimental set-ups with these cell lines.
Keywords:
ADPKD; Calcium; Human cell lines; cAMP; mTOR.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Antidiuretic Hormone Receptor Antagonists / pharmacology
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Antidiuretic Hormone Receptor Antagonists / therapeutic use
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Calcium Signaling
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Cell Line
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Cell Proliferation
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Cilia / pathology
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Clinical Trials as Topic
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Glucosidases / genetics
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Humans
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Kidney / cytology
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Kidney / drug effects
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Kidney / pathology
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Kidney / physiopathology*
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Mutation
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Polycystic Kidney, Autosomal Dominant / drug therapy
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Polycystic Kidney, Autosomal Dominant / genetics
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Polycystic Kidney, Autosomal Dominant / pathology
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Polycystic Kidney, Autosomal Dominant / physiopathology*
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Primary Cell Culture / methods
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TOR Serine-Threonine Kinases / metabolism
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TRPP Cation Channels / genetics
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Tolvaptan / pharmacology
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Tolvaptan / therapeutic use
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Treatment Outcome
Substances
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Antidiuretic Hormone Receptor Antagonists
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TRPP Cation Channels
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polycystic kidney disease 1 protein
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polycystic kidney disease 2 protein
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Tolvaptan
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MTOR protein, human
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TOR Serine-Threonine Kinases
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GANAB protein, human
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Glucosidases