Jitt # 7 APECED Flashcards Preview

Medical Immunology Bios 443/843 > Jitt # 7 APECED > Flashcards

Flashcards in Jitt # 7 APECED Deck (9)
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1
Q

APECED

A

Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (Also known as APSI); human autosomal recessive disease; Mutation in AIRE (which is not encoded by MHC genes);

2
Q

What cell types play the most important role in negative selection?

A

Bone-Marrow derived DCs and Macrophages; Thymic medullary epithelial cells also play a role.

3
Q

Symptoms of APECED

A

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;

4
Q

Which cytokines do patients with APECED have antibodies against?

A

IL-17A, IL-17E, and IL-22. These are important cytokines in controlling fungal infections.

5
Q

Negative selection

A

Single Positive thymocytes who bind to antigens in the thymic medulla with high avidity undergo apoptosis.

6
Q

Central Tolerance

A

Induced when developing
lymphocytes encounter self antigens in the
primary or generative lymphoid organs (thymus
and bone marrow)

7
Q

Peripheral Tolerance

A

Induced when mature lymphocytes encounter self antigens in peripheral tissues.

8
Q

What is the fate of thymocytes that bind with intermediate affinity?

A

They become Treg. Regulatory T Cells.

9
Q

Generation of Treg

A

Occurs in the thymus; Thymocytes bind with intermediate affinity; Transcription factor FoxP3 upregulated; Treg moves to the periphery and inhibits self reactive T cells.

Decks in Medical Immunology Bios 443/843 Class (56):