A new epitope-tagged Pkhd1 allele sheds light on fibrocystin signaling.

Lea, Wendy A; Ward, Christopher J · Kidney Int · 2017

other · Level V

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Abstract

In this issue of Kidney International, Outeda et al. present a new epitope-tagged allele of murine Pkhd1 that allows the monitoring of functional fibrocystin in vivo from the extreme C-terminus of the molecule. This work also shows that the removal of two-thirds of the intracellular tail of fibrocystin does not result in cystogenesis in either the liver or kidney, with major implications for our understanding of Pkhd1 function and polycystic kidney disease in general.

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