Ontology highlight
ABSTRACT:
SUBMITTER: Legembre P
PROVIDER: S-EPMC1299175 | biostudies-literature | 2004 Nov
REPOSITORIES: biostudies-literature
Legembre Patrick P Barnhart Bryan C BC Zheng Lixin L Vijayan Shrijay S Straus Stephen E SE Puck Jennifer J Dale Janet K JK Lenardo Michael M Peter Marcus E ME
EMBO reports 20041101 11
Mutations in the death domain of the death receptor CD95 (APO-1/Fas) cause lymphoproliferation and autoimmune disease in both lpr(cg) mice and in patients with autoimmune lymphoproliferative syndrome (ALPS) type Ia. By testing lymphocytes from ALPS type Ia patients, comparing heterozygous with homozygous lpr(cg) mice and coexpressing wild-type and mutant CD95 receptors, we demonstrate that induction of apoptosis requires two wild-type alleles of CD95. By contrast, nuclear factor-kappaB (NF-kappa ...[more]