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Fas preassociation required for apoptosis signaling and dominant inhibition by pathogenic mutations.

Publication ,  Journal Article
Siegel, RM; Frederiksen, JK; Zacharias, DA; Chan, FK; Johnson, M; Lynch, D; Tsien, RY; Lenardo, MJ
Published in: Science
June 30, 2000

Heterozygous mutations encoding abnormal forms of the death receptor Fas dominantly interfere with Fas-induced lymphocyte apoptosis in human autoimmune lymphoproliferative syndrome. This effect, rather than depending on ligand-induced receptor oligomerization, was found to stem from ligand- independent interaction of wild-type and mutant Fas receptors through a specific region in the extracellular domain. Preassociated Fas complexes were found in living cells by means of fluorescence resonance energy transfer between variants of green fluorescent protein. These results show that formation of preassociated receptor complexes is necessary for Fas signaling and dominant interference in human disease.

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Published In

Science

DOI

ISSN

0036-8075

Publication Date

June 30, 2000

Volume

288

Issue

5475

Start / End Page

2354 / 2357

Location

United States

Related Subject Headings

  • fas Receptor
  • Tumor Cells, Cultured
  • Succinimides
  • Signal Transduction
  • Recombinant Fusion Proteins
  • Point Mutation
  • Mutation
  • Mice
  • Membrane Glycoproteins
  • Macromolecular Substances
 

Citation

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Siegel, R. M., Frederiksen, J. K., Zacharias, D. A., Chan, F. K., Johnson, M., Lynch, D., … Lenardo, M. J. (2000). Fas preassociation required for apoptosis signaling and dominant inhibition by pathogenic mutations. Science, 288(5475), 2354–2357. https://doi.org/10.1126/science.288.5475.2354
Siegel, R. M., J. K. Frederiksen, D. A. Zacharias, F. K. Chan, M. Johnson, D. Lynch, R. Y. Tsien, and M. J. Lenardo. “Fas preassociation required for apoptosis signaling and dominant inhibition by pathogenic mutations.Science 288, no. 5475 (June 30, 2000): 2354–57. https://doi.org/10.1126/science.288.5475.2354.
Siegel RM, Frederiksen JK, Zacharias DA, Chan FK, Johnson M, Lynch D, et al. Fas preassociation required for apoptosis signaling and dominant inhibition by pathogenic mutations. Science. 2000 Jun 30;288(5475):2354–7.
Siegel, R. M., et al. “Fas preassociation required for apoptosis signaling and dominant inhibition by pathogenic mutations.Science, vol. 288, no. 5475, June 2000, pp. 2354–57. Pubmed, doi:10.1126/science.288.5475.2354.
Siegel RM, Frederiksen JK, Zacharias DA, Chan FK, Johnson M, Lynch D, Tsien RY, Lenardo MJ. Fas preassociation required for apoptosis signaling and dominant inhibition by pathogenic mutations. Science. 2000 Jun 30;288(5475):2354–2357.
Journal cover image

Published In

Science

DOI

ISSN

0036-8075

Publication Date

June 30, 2000

Volume

288

Issue

5475

Start / End Page

2354 / 2357

Location

United States

Related Subject Headings

  • fas Receptor
  • Tumor Cells, Cultured
  • Succinimides
  • Signal Transduction
  • Recombinant Fusion Proteins
  • Point Mutation
  • Mutation
  • Mice
  • Membrane Glycoproteins
  • Macromolecular Substances