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In Vitro and In Vivo Models to Study Nephropathic Cystinosis.

Cells - 21 Dec 2021

Cheung Pang Yuk, Harrison Patrick T, Davidson Alan J, Hollywood Jennifer A

Abstract excerpt

The development over the past 50 years of a variety of cell lines and animal models has provided valuable tools to understand the pathophysiology of nephropathic cystinosis. Primary cultures from patient biopsies have been instrumental in determining the primary cause of cystine accumulation in the lysosomes. Immortalised cell lines have been established using different gene constructs and have revealed a wealth...

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PMID: 35011573
DOI: 10.3390/cells11010006
PMCID: PMC8750259

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