Fas Preassociation Required for Apoptosis Signaling and Dominant Inhibition by Pathogenic Mutations

摘要: 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 oligomerization, was found to stem from ligand- independent interaction wild-type and mutant receptors through a specific region extracellular domain. Preassociated complexes were living cells by means fluorescence resonance energy transfer between variants green fluorescent protein. These results show that formation preassociated is necessary for signaling dominant interference disease.