Primary immunodeficiency diseases: natural mutant models for the study of the immune system.

Abstract excerpt

Primary immunodeficiency diseases represent many natural mutant models for the analysis of many aspects of immunology. Examples dealing with T- and B-cell receptors are herein discussed. They also represent privileged models in medicine, because well characterized monogenic inherited diseases can in some instances be viewed as good targets for gene therapy as shown here for severe combined immunodeficiency (SCID).

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