Ontology highlight
ABSTRACT:
SUBMITTER: Wang L
PROVIDER: S-EPMC2988912 | biostudies-literature | 2010 Nov
REPOSITORIES: biostudies-literature
Nature structural & molecular biology 20101010 11
The death-inducing signaling complex (DISC) formed by the death receptor Fas, the adaptor protein FADD and caspase-8 mediates the extrinsic apoptotic program. Mutations in Fas that disrupt the DISC cause autoimmune lymphoproliferative syndrome (ALPS). Here we show that the Fas-FADD death domain (DD) complex forms an asymmetric oligomeric structure composed of 5-7 Fas DD and 5 FADD DD, whose interfaces harbor ALPS-associated mutations. Structure-based mutations disrupt the Fas-FADD interaction in ...[more]