Article

Cystin is required for maintaining fibrocystin (FPC) levels and safeguarding proteome integrity in mouse renal epithelial cells A mechanistic connection between the kidney defects in <i>cpk</i> mice and human ARPKD

2022-04-19

Abstract excerpt

<h4>ABSTRACT</h4> Autosomal recessive polycystic kidney disease (ARPKD) is caused primarily by mutations in PKHD1 , encoding fibrocystin (FPC), but Pkhd1 mutant mice fail to express renal cystic disease. In contrast, the renal lesion in Cys1 cpk/cpk ( cpk ) mice with loss of the cystin protein, closely phenocopy ARPKD. Recent identification of patients with CYS1 -related ARPKD prompted the investigations des...

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Literature Corpus work: 42b449c3-9da9-58c3-99af-cdc2c9a66891
DOI: 10.1101/2022.04.19.488799

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