Immunodeficiency Syndromes Flashcards
Defects in T cell function predispose host to IC (viral) infections. True or false?
True
What do defects in B cell function and Ig production predispose animals to?
EC bacterial disease
Why are combined immunodeficiences severe?
Because affected animals lack resistance to ALL infectious agents
What is the difference between congenital and acquired immunodeficiencies?
Congenital (primary) affects the innate and adaptive immune system and is genetic.
Acquired (secondary) is cause by microbial infections.
How are genetic disorders inherited?
Autosomal recessive inheritance happens when parents carry the gene defect.
X-Linked inheritance occurs as a result of mutations in the X chromosome.
The is no male to male transmission in X-linked inheritance. True or false?
True
Leukocyte adhesion deficiency type I- innate or adaptive disorder?
innate, autosomal
What molecule is affected in leukocyte adhesion deficiency type I and why is this critical?
CD18 becomes non-functional.
CD18 is the integrin critical for leukocyte adhesion and migration into the tissues, when it is non-functional leukocytes cannot migrate to site of infection.
What are Bovine LAD and Canine LAD?
leukocyte adhesion deficiency type I affecting cattle and dogs
What molecule is affected in leukocyte adhesion deficiency type II and why is this critical?
Defect is in L-selectin.
Selectin molecules interact with leukocytes to slow them down to allow them to migrate into infected tissues.
Chediak-Higashi syndrome- innate or adaptive disease?
innate, autosomal
What molecule is affected in Chediak-Higashi syndrome and why is this critical?
LYST is affected
Prevents lysosome from fusing to phagosome containing trapped pathogen.
What cells are affected by Chediak-Higashi syndrome?
neutrophils, macrophages, DCs, NK cells, cytotoxic T cells, etc
What species are most affected by Chediak-Higashi syndrome?
cats and cattle
What are the consequences of Chediak-Higashi syndrome?
susceptibility to diseases and early death
What is the affected molecule in Chronic granulomatous disease and why is it critical? Innate or adaptive?
Innate
Phagocyte oxidase complex Phox-19 affected. Prevents the phagoytes from making reactive oxygen species making them useless because that is their killing mechism.
What is affected in TLR signaling defects?
CD40
PAMPs cannot be sensed, resulting in host being unable to signal the rest of the body that there is an active infection
Complement deficiences make up what percentage of all primary immunodeficiencies?
1-10%