RAGs-associated SCID

T cell-negative (T-), B cell-negative (B-), natural killer cell-positive (NK+) severe combined immunodeficiency (SCID) can be caused by mutation in the recombinase activating genes RAG1 (MIM.179615) and RAG2 (MIM.179616).

Nota bene: Omenn syndrome (MIM.603554) is an immunodeficient disorder with a less severe phenotype, also caused by mutation in the RAG1 and RAG2 genes.

See also

 T-, B-, NK+ SCID with sensitivity to ionizing radiation (MIM.602450)

• caused by mutation in the DCLRE1C-Artemis gene (DCLRE1C; MIM.605988)

 T-, B-, NK+ SCID with microcephaly, growth retardation, and sensitivity to ionizing radiation (MIM.611291)

• caused by mutation in the NHEJ1 gene (MIM.611290).