Analysis of Primary Immunodeficiency Syndromes using model mice.

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We tried to generate Severe Congenital Neutropenia (SCN) model mouse. We produced wild-type (WT), P110L, R101Q and C194X ELA2 by PCR-based mutagenesis and introduced them into retrovirus Tet-on gene expression vector. We performed gene expression experiment by using HEK293 cells. Although ELA2 expression was observed, we could not analyze them because the ELA2 introduced cells result in cell death. Now, we are trying to control ELA2 expression level by control the concentration of doxycycline. After that, we are planning generate SCN model mouse by BM reconstitution using CD34 positive cells from human cord blood.