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

Brittney-Shea Herbert, Brenda Grimes, Wei Min Xu, Michael Werner, Christopher Ward, Sandro Rossetti, Peter Harris, Elsa Bello-Reuss, Heather H. Ward, Caroline Miller, Vincent H. Gattone, Carrie Phillips, Angela Wandinger-Ness, Robert Bacallao

Research output: Contribution to journalArticle

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Abstract

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.

Original languageEnglish
Article numbere55191
JournalPLoS One
Volume8
Issue number1
DOIs
StatePublished - Jan 28 2013

Fingerprint

telomerase
proximal tubules
Telomerase
Cells
cell lines
mutation
Autosomal Dominant Polycystic Kidney
Cell Line
Mutation
Cilia
cilia
Kidney
Cysts
kidneys
cells
Cell culture
nephrons
Epithelial-Mesenchymal Transition
Nephrons
kidney cells

ASJC Scopus subject areas

  • Agricultural and Biological Sciences(all)
  • Biochemistry, Genetics and Molecular Biology(all)
  • Medicine(all)

Cite this

A Telomerase Immortalized Human Proximal Tubule Cell Line with a Truncation Mutation (Q4004X) in Polycystin-1. / Herbert, Brittney-Shea; Grimes, Brenda; Xu, Wei Min; Werner, Michael; Ward, Christopher; Rossetti, Sandro; Harris, Peter; Bello-Reuss, Elsa; Ward, Heather H.; Miller, Caroline; Gattone, Vincent H.; Phillips, Carrie; Wandinger-Ness, Angela; Bacallao, Robert.

In: PLoS One, Vol. 8, No. 1, e55191, 28.01.2013.

Research output: Contribution to journalArticle

Herbert, B-S, Grimes, B, Xu, WM, Werner, M, Ward, C, Rossetti, S, Harris, P, Bello-Reuss, E, Ward, HH, Miller, C, Gattone, VH, Phillips, C, Wandinger-Ness, A & Bacallao, R 2013, 'A Telomerase Immortalized Human Proximal Tubule Cell Line with a Truncation Mutation (Q4004X) in Polycystin-1', PLoS One, vol. 8, no. 1, e55191. https://doi.org/10.1371/journal.pone.0055191
Herbert, Brittney-Shea ; Grimes, Brenda ; Xu, Wei Min ; Werner, Michael ; Ward, Christopher ; Rossetti, Sandro ; Harris, Peter ; Bello-Reuss, Elsa ; Ward, Heather H. ; Miller, Caroline ; Gattone, Vincent H. ; Phillips, Carrie ; Wandinger-Ness, Angela ; Bacallao, Robert. / A Telomerase Immortalized Human Proximal Tubule Cell Line with a Truncation Mutation (Q4004X) in Polycystin-1. In: PLoS One. 2013 ; Vol. 8, No. 1.
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