A Telomerase Immortalized Human Proximal Tubule Cell Line with a Truncation Mutation (Q4004X) in Polycystin-1 | Zendy

BrittneyShea Herbert | Zendy; Brenda R. Grimes | Zendy; Wei Xu | Zendy; Michael E. Werner | Zendy; Christopher J. Ward | Zendy; Sandro Rossetti | Zendy; Peter C. Harris | Zendy; Elsa BelloReuss | Zendy; Heather Ward | Zendy; Caroline A. Miller | Zendy; Vincent H. Gattone | Zendy; Carrie L. Phillips | Zendy; Angela WandingerNess | Zendy; Robert L. Bacallao | Zendy

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A Telomerase Immortalized Human Proximal Tubule Cell Line with a Truncation Mutation (Q4004X) in Polycystin-1

Author(s) -

BrittneyShea Herbert,

Brenda R. Grimes,

Wei Xu,

Michael E. Werner,

Christopher J. Ward,

Sandro Rossetti,

Peter C. Harris,

Elsa BelloReuss,

Heather Ward,

Caroline A. Miller,

Vincent H. Gattone,

Carrie L. Phillips,

Angela WandingerNess,

Robert L. Bacallao

Publication year - 2013

Publication title -

plos one

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.99

H-Index - 332

ISSN - 1932-6203

DOI - 10.1371/journal.pone.0055191

Subject(s) - cilium , telomerase , immortalised cell line , autosomal dominant polycystic kidney disease , microbiology and biotechnology , biology , cell culture , nephron , epithelium , ciliogenesis , telomerase reverse transcriptase , kidney , cell , cyst , mutation , pathology , endocrinology , medicine , genetics , gene

Autosomal dominant polycystic kidney disease (ADPKD) is associated with a variety of cellular phenotypes in renal epithelial cells. Cystic epithelia are secretory as opposed to absorptive, have higher proliferation rates in cell culture and have some characteristics of epithelial to mesenchymal transitions [1] , [2] . In this communication we describe a telomerase immortalized cell line that expresses proximal tubule markers and is derived from renal cysts of an ADPKD kidney. These cells have a single detectable truncating mutation (Q4004X) in polycystin-1. These cells make normal appearing but shorter cilia and fail to assemble polycystin-1 in the cilia, and less uncleaved polycystin-1 in membrane fractions. This cell line has been maintained in continuous passage for over 35 passages without going into senescence. Nephron segment specific markers suggest a proximal tubule origin for these cells and the cell line will be useful to study mechanistic details of cyst formation in proximal tubule cells.

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