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Defective potassium currents in ataxia telangiectasia fibroblasts.

Publication ,  Journal Article
Rhodes, N; D'Souza, T; Foster, CD; Ziv, Y; Kirsch, DG; Shiloh, Y; Kastan, MB; Reinhart, PH; Gilmer, TM
Published in: Genes Dev
December 1, 1998

Similarities exist between the progressive cerebellar ataxia in ataxia telangiectasia (AT) patients and a number of neurodegenerative diseases in both mouse and man involving specific mutations in ion channels and/or ion channel activity. These relationships led us to investigate the possibility of defective ion channel activity in AT cells. We examined changes in the membrane potential of AT fibroblasts in response to extracellular cation addition and found that the ability of AT fibroblasts to depolarize in response to increasing concentrations of extracellular K+ is significantly reduced when compared with control fibroblasts. Electrophysiological measurements performed with a number of AT cell lines, as well as two matched sets of primary AT fibroblast cultures, reveal that outward rectifier K+ currents are largely absent in AT fibroblasts in comparison with control cells. These K+ current defects can be corrected in AT fibroblasts transfected with the full-length ATM cDNA. These data implicate, for the first time, a role for ATM in the regulation of K+ channel activity and membrane potential.

Duke Scholars

Published In

Genes Dev

DOI

ISSN

0890-9369

Publication Date

December 1, 1998

Volume

12

Issue

23

Start / End Page

3686 / 3692

Location

United States

Related Subject Headings

  • Potassium Channels
  • Potassium
  • Membrane Potentials
  • Humans
  • Fibroblasts
  • Electric Conductivity
  • Developmental Biology
  • Cell Line
  • Ataxia Telangiectasia
  • 52 Psychology
 

Citation

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ICMJE
MLA
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Rhodes, N., D’Souza, T., Foster, C. D., Ziv, Y., Kirsch, D. G., Shiloh, Y., … Gilmer, T. M. (1998). Defective potassium currents in ataxia telangiectasia fibroblasts. Genes Dev, 12(23), 3686–3692. https://doi.org/10.1101/gad.12.23.3686
Rhodes, N., T. D’Souza, C. D. Foster, Y. Ziv, D. G. Kirsch, Y. Shiloh, M. B. Kastan, P. H. Reinhart, and T. M. Gilmer. “Defective potassium currents in ataxia telangiectasia fibroblasts.Genes Dev 12, no. 23 (December 1, 1998): 3686–92. https://doi.org/10.1101/gad.12.23.3686.
Rhodes N, D’Souza T, Foster CD, Ziv Y, Kirsch DG, Shiloh Y, et al. Defective potassium currents in ataxia telangiectasia fibroblasts. Genes Dev. 1998 Dec 1;12(23):3686–92.
Rhodes, N., et al. “Defective potassium currents in ataxia telangiectasia fibroblasts.Genes Dev, vol. 12, no. 23, Dec. 1998, pp. 3686–92. Pubmed, doi:10.1101/gad.12.23.3686.
Rhodes N, D’Souza T, Foster CD, Ziv Y, Kirsch DG, Shiloh Y, Kastan MB, Reinhart PH, Gilmer TM. Defective potassium currents in ataxia telangiectasia fibroblasts. Genes Dev. 1998 Dec 1;12(23):3686–3692.

Published In

Genes Dev

DOI

ISSN

0890-9369

Publication Date

December 1, 1998

Volume

12

Issue

23

Start / End Page

3686 / 3692

Location

United States

Related Subject Headings

  • Potassium Channels
  • Potassium
  • Membrane Potentials
  • Humans
  • Fibroblasts
  • Electric Conductivity
  • Developmental Biology
  • Cell Line
  • Ataxia Telangiectasia
  • 52 Psychology