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Tuberin-dependent membrane localization of polycystin-1: a functional link between polycystic kidney disease and the TSC2 tumor suppressor gene.

Publication ,  Journal Article
Kleymenova, E; Ibraghimov-Beskrovnaya, O; Kugoh, H; Everitt, J; Xu, H; Kiguchi, K; Landes, G; Harris, P; Walker, C
Published in: Mol Cell
April 2001

The PKD1 gene accounts for 85% of autosomal dominant polycystic kidney disease (ADPKD), the most common human genetic disorder. Rats with a germline inactivation of one allele of the Tsc2 tumor suppressor gene developed early onset severe bilateral polycystic kidney disease, with similarities to the human contiguous gene syndrome caused by germline codeletion of PKD1 and TSC2 genes. Polycystic rat renal cells retained two normal Pkd1 alleles but were null for Tsc2 and exhibited loss of lateral membrane-localized polycystin-1. In tuberin-deficient cells, intracellular trafficking of polycystin-1 was disrupted, resulting in sequestration of polycystin-1 within the Golgi and reexpression of Tsc2 restored correct polycystin-1 membrane localization. These data identify tuberin as a determinant of polycystin-1 functional localization and, potentially, ADPKD severity.

Duke Scholars

Published In

Mol Cell

DOI

ISSN

1097-2765

Publication Date

April 2001

Volume

7

Issue

4

Start / End Page

823 / 832

Location

United States

Related Subject Headings

  • Tumor Suppressor Proteins
  • Tumor Cells, Cultured
  • Tuberous Sclerosis Complex 2 Protein
  • Transfection
  • TRPP Cation Channels
  • Repressor Proteins
  • Rats
  • Proteins
  • Kidney Neoplasms
  • Golgi Apparatus
 

Citation

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Kleymenova, E., Ibraghimov-Beskrovnaya, O., Kugoh, H., Everitt, J., Xu, H., Kiguchi, K., … Walker, C. (2001). Tuberin-dependent membrane localization of polycystin-1: a functional link between polycystic kidney disease and the TSC2 tumor suppressor gene. Mol Cell, 7(4), 823–832. https://doi.org/10.1016/s1097-2765(01)00226-x
Kleymenova, E., O. Ibraghimov-Beskrovnaya, H. Kugoh, J. Everitt, H. Xu, K. Kiguchi, G. Landes, P. Harris, and C. Walker. “Tuberin-dependent membrane localization of polycystin-1: a functional link between polycystic kidney disease and the TSC2 tumor suppressor gene.Mol Cell 7, no. 4 (April 2001): 823–32. https://doi.org/10.1016/s1097-2765(01)00226-x.
Kleymenova E, Ibraghimov-Beskrovnaya O, Kugoh H, Everitt J, Xu H, Kiguchi K, et al. Tuberin-dependent membrane localization of polycystin-1: a functional link between polycystic kidney disease and the TSC2 tumor suppressor gene. Mol Cell. 2001 Apr;7(4):823–32.
Kleymenova, E., et al. “Tuberin-dependent membrane localization of polycystin-1: a functional link between polycystic kidney disease and the TSC2 tumor suppressor gene.Mol Cell, vol. 7, no. 4, Apr. 2001, pp. 823–32. Pubmed, doi:10.1016/s1097-2765(01)00226-x.
Kleymenova E, Ibraghimov-Beskrovnaya O, Kugoh H, Everitt J, Xu H, Kiguchi K, Landes G, Harris P, Walker C. Tuberin-dependent membrane localization of polycystin-1: a functional link between polycystic kidney disease and the TSC2 tumor suppressor gene. Mol Cell. 2001 Apr;7(4):823–832.
Journal cover image

Published In

Mol Cell

DOI

ISSN

1097-2765

Publication Date

April 2001

Volume

7

Issue

4

Start / End Page

823 / 832

Location

United States

Related Subject Headings

  • Tumor Suppressor Proteins
  • Tumor Cells, Cultured
  • Tuberous Sclerosis Complex 2 Protein
  • Transfection
  • TRPP Cation Channels
  • Repressor Proteins
  • Rats
  • Proteins
  • Kidney Neoplasms
  • Golgi Apparatus