Article
Expression in transgenic mice of dominant interfering Fas mutations: a model for human autoimmune lymphoproliferative syndrome.
Clinical immunology (Orlando, Fla.) - 1 Oct 1999
Choi Y, Ramnath V R, Eaton A S, Chen A, Simon-Stoos K L, Kleiner D E, Erikson J, Puck J M
Abstract excerpt
Most humans with autoimmune lymphoproliferative syndrome (ALPS) carry heterozygous dominant mutations in one allele of the gene encoding Fas/APO-1/CD95. ALPS patients, like Fas-deficient MRL lpr/lpr mice, have lymphoproliferation, autoimmunity, increased CD4(-)/CD8(-) T lymphocytes, and apoptosis defects. Consistent with the phenotypic variability of lpr/lpr mice of different background strains, human genetic...
Topics
- Animals
- Antibodies, Antinuclear
- Antigens, Surface
- Apoptosis
- Autoimmune Diseases
- Fas Ligand Protein
- Genes, Dominant
- Humans
- Liver
- Liver Diseases
- Lymph Nodes
- Lymphatic Diseases
- Lymphoproliferative Disorders