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

Journal Article (Journal Article)

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.

Full Text

Duke Authors

Cited Authors

  • Siegel, RM; Frederiksen, JK; Zacharias, DA; Chan, FK; Johnson, M; Lynch, D; Tsien, RY; Lenardo, MJ

Published Date

  • June 30, 2000

Published In

Volume / Issue

  • 288 / 5475

Start / End Page

  • 2354 - 2357

PubMed ID

  • 10875918

International Standard Serial Number (ISSN)

  • 0036-8075

Digital Object Identifier (DOI)

  • 10.1126/science.288.5475.2354


  • eng

Conference Location

  • United States