Specifically B cell or T cell Both: SCID ( B and T cells) XLA: All IgM, IgG.

Why Abs impo: neutralize, Fc receptor, complement recptors: to be phaygocytosed Eny Tyr kinase, Btk Rearrange genes for diverse Abs Heavy chain genes ->L-chain Btk for signal of heavychain: dont know if H-chain rearranged, wont proliferate Mom: mutant(white on one X: 50% son affected) Treatment: passive immunity injection every 3-4 wks BLS BLS I and II: MHC class I or II MHC class deficienty, class I: less severe, can survive quite well Class I: in ER, peptides degraded thru protesome, smaller fragments translocated into ER, (transporter) classII: like SCID, no functional B or T cell response absence of CD4: orchestra, impo for amounting humonral AND cellular mutation of TF to activate MHC class I expression lack of T cell development: need to interact with MHC II think they cant recognize BLSI: CD8 T cell, II: CD4- die by neglect BLSII SCID, bubble x-linked: Lack of T cell lineage and NK cells-cellular and humonral immunity: atrophy of thymus, IL-2 x, IL-7 T cell development, Pro-T need IL-7 signal, for trigger of further differentiation NK: IL-15, innate, first cell for viral infectionsinitial innate immunity Autosomal SCID: progressive loss Enzyme deficiency B and T cells disappear So far, dec number Lymphocyte activation: normal number, but still immunife deficiency Hyper IgM, dec IgG, igA, igE, and inc IgM dec cellular and humoral

Bad opsonization, more susceptible to pyrogenic CD 40 ligand: interact with CD40 and macrophage: cellular when T cell activated Isotype switching x: high aff Abs by B cells Def in innate immunue Cant get to the site, elevated in the bloodstream Inability of leukocyte to recognize ligand, underlying endothelium, to adhere endothelium Mutation in gene: CD 18(beta2) NOT 11 Macrophage cant express integrins, T cells: some function Complement pathway: series of proteins into bloodstream, bind to Abs, Using receptors on macrophage, Inflame C2 and 4: classical pathway: symptoms like autoimmune dz(SLE) Small Ab and Ag complex, clog Need binding of complement to phagocytosed Terminal compo(autosommal, C5-9) more susceptible to nessiria bacteria