Flashcards in Jitt # 7 APECED Deck (9):
Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (Also known as APSI); human autosomal recessive disease; Mutation in AIRE (which is not encoded by MHC genes);
What cell types play the most important role in negative selection?
Bone-Marrow derived DCs and Macrophages; Thymic medullary epithelial cells also play a role.
Symptoms of APECED
Decreased growth; inadequate growth hormone; high levels of TSH; antibodies against endocrine glands, liver, and skin; increased susceptibility to infection with candida albicans; abnormalities in skin, teeth and fingernails;
Which cytokines do patients with APECED have antibodies against?
IL-17A, IL-17E, and IL-22. These are important cytokines in controlling fungal infections.
Single Positive thymocytes who bind to antigens in the thymic medulla with high avidity undergo apoptosis.
Induced when developing
lymphocytes encounter self antigens in the
primary or generative lymphoid organs (thymus
and bone marrow)
Induced when mature lymphocytes encounter self antigens in peripheral tissues.
What is the fate of thymocytes that bind with intermediate affinity?
They become Treg. Regulatory T Cells.