Autoimmunity Flashcards
What is the definition of autoimmunity
The presence of immune responses against self-antigens
How are antibodies generated
antibodies are generated by the production of heavy chain and light chain polypeptides that are synthesized from two separate immunoglobulin genes.
what are heavy and light chains composed of?
segmented genes
What do regulatory T cells help do?
Regulatory T cells can help inactivate auto-reactive lymphocytes in peripheral tissues that escape central tolerance. (peripheral tolerance).
Which regulatory cells are crucial for the suppressing hyper-reactive or auto-reactive T cells.
Regulatory T cells.
What are monogenic disorders
single gene defects causing autoimmune diseases.
What does IPEX stand for
Immune dysregulation, polyendocrinopathy, enteropathy and X-linked inheritance syndrome.
What is IPEX syndrome.
A rare genetic disorder of immune dysregulation.
What are the symptoms of IPEX syndrome
Severe infections
intractable diarrhoea
eczema
very clearly onset insulin dependant diabetes mellitus
autoimmune manifestations
What is the cure for IPEX syndrome
hematopoietic stem cell transplantations (HSCT)
What is the pathogenesis of IPEX syndrome.
condition is X-linked, caused by mutations the FOXP3 gene, hence IPEX causes a failure of peripheral tolerance mechanisms due to the absence regulatory T cells.
What does HLA stand for
Human Leucocyte Antigen
Which environmental factors can trigger autoimmunity in genetically predisposed individuals.
infections, cigarette smoking, hormone levels, tissue damage.
What are mechanisms of autoimmune disease
molecular mimicry
alterations to self-antigens
antigen sequestration
bacterial superantigens
