affix antiget to surface> specific antibody is washed over surface to bind to antigen, antibody linked to enzyme, substance is added that converts enzyme to detectable signal, such as fluoresence

autosomal receessive

mutatoin in LYST

impaired bacteriolysis in failure of phagolysosome formation> impaired lysosome degranulation with phagosomes > no phagocytozed bacteria destroyed by lysossomes enzymes

functional immune cells in transplanted marrow recognize recipient as foreign and mount immunologic attack

T cells in graft recognize new host as foriegn and mount attack. T cells produce excess cytokines (inc TNFalpha and INF gamma)

defect in FAS-FAS-L induction of apotosis

enlarged lymphoid organs as result of over populatioin of T and B cells

Leucocyte adhesion deficiency

deficiency of Beta2 integrin subunit > CD18

subunit is involved in 3 other proteins > LFA1, Integrin alphaXbeta2, and MAC1- CD3

gene creates non functioning protein

basically leucocytes cannot migrate to the infection as part of the inflammatory response

severe combined immunodeficiency

SCID

improper function of entire adaptive immune system

caused by one of many failures in the development of T and B cells

examples include

RAG genes - regulate random recombination of genes for development of repetoire

X linked > deficiency in common gamma chain of IL-2R subfamily

bradykinin

vasc perm

vasodilation

pain- prostigland increase

pain- smooth muscle contraction

target is vascular endothelium

more exudate

comp activation

stop inflammatory response

FAS expressed on target cell > induce apoptosis

apototic cell will be phagocyotized

take home > FAS ligand apoptosis is for inflammation

CD8- CD4- double negative is just for some reason a marker for FAS-FASL defect of apotosis - ALPS