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Article

A novel model of autosomal recessive polycystic kidney questions the role of the fibrocystin C-terminus in disease mechanism.

Kidney international - 1 Nov 2017

Outeda Patricia, Menezes Luis, Hartung Erum A, Bridges Stacey, Zhou Fang, Zhu Xianjun, Xu Hangxue, Huang Qiong, Yao Qin, Qian Feng, Germino Gregory G, Watnick Terry

Abstract excerpt

Autosomal recessive polycystic kidney disease (OMIM 263200) is a serious condition of the kidney and liver caused by mutations in a single gene, PKHD1. This gene encodes fibrocystin/polyductin (FPC, PD1), a large protein shown by in vitro studies to undergo Notch-like processing. Its cytoplasmic tail, reported to include a ciliary targeting sequence, a nuclear localization signal, and a polycystin-2 binding...

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Source

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Identifiers

PMID: 28729032
DOI: 10.1016/j.kint.2017.04.027
PMCID: PMC6005173

Genes

PKHD1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

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