This compound mutant strain carries deletions in the mouse Rag2 and X-linked Il2rg genes in addition to targeted replacements of mouse Il3 (interleukin 3) and Csf2 (colony stimulating factor 2 (granulocyte-macrophage); GM-CSF) coding regions with those of human.

Homozygotes develop pulmonary alveolar proteinosis and are susceptible to opportunistic lung infections due to a defect in the terminal differentiation of alveolar macrophages (similar to Csf2-deficient mice). The concentration level and tissue distribution pattern of both human IL3 and CSF2 expression mimic those of the mouse genes. The Rag2 and Il2rg mutations create an immunodeficiency in which T, B, and NK cells are lacking. 

These mice are particularly useful as graft/transplant hosts that support human myeloid cell reconstitution and function as well as human innate immune responses to pathogens.

The creation of this strain was funded by the Bill and Melinda Gates Foundation.

This immunodeficient strain carrying humanized IL3 and CSF2 (GM-CSF) cytokines may be useful for studies of lung disease and infection.

Typically mice are recovered in 12-16 weeks. Contact Customer Service to place an order or for more information.

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