Microbiology and Immunology 3300B Lecture Notes - Lecture 17: Jak 3, Chemotherapy, Retrovirus
20 May 2016
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The most severe of primary immunodeficiencies: primary = genetic. Unless treated, scid is lethal before the childs first or second birthday. Classified by the detection of t, b and nk cells. Bbay joe had a mutation in his rag gene: mutation in his rag gene resulted in no recombination, no b cell receptor generated. Immune deficiencies are defects of development: myeloid or lymphoid development, everything downstream the defect will be effected, scid developments happen early. Can effect lymphoid progenitor cell, pre b cell. Gamma c is the most common cause of skid. Developing t cell progenitors also respond il 7, not just pro b cells. If il7 is mutated, b cells are still present but do not become pro b cells. Il 7 receptor requires jak3 to phosphorylate stat to transcribe essential genes for t cell development. Gammac cytokines signal through a common jak3 mechanism. Many receptors for different cytokines use gamma c, that"s why it"s the most common mutation.
