Figure 4 Association between the TCR-CD3 complex and ZAP-70.

has led to the cloning of MHC class II transactivator (CIITA), X box binding factor regulatory factor X (RFX5), and RFXAP genes. Group A is caused by mutations of the CIITA gene, group C is caused by mutations of RFX5 and group D is caused by mutations of RFXAP. The gene responsible for group B has not yet been identified. Some patients with MHC class II deficiency also have decreased expression of MHC class I for unknown reasons.

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