Lineage-specific expression of CYBB in leukocytes

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(1) Roles of PU.1 and HAF-1 for the expression of CYBBSingle base substitutions were systematically induced to the CYBB promoter and examined their effects on reporter activities and binding affinities to PU.1 and HAF-1. Data suggested that PU.1 is principal and HAF-1 supplementary. These also implied new CGDs and supplied ideally modified promoter sequences for maximal expression of the gene in the CGD phagocytes.(2) A new cw-elemement for the activation of CYBBWe discovered a new GATA-binding site on the CYBB promoter. This site is essential for the expression of the gene promoted by PU.1 and GATA-1.(3) A role of IRF-4 in B lymphocytesIRF-4 was suggested to inhibit CYBB that may account for the low expression of gp91phox in B lymphocytes.(4) Trials for GATA-1 gene transfection to phagocytes of the X-CGD patient with an abnormal CYBB promoterFor making gp91phox-deficient neutrophils/monocytes to gp91phox-normal eosinophils, we tried to transfect GATA-1 gene to the peripheral blood monocytes of a patient with X-linked bp-53T CGD patient, Efficiencies of liposomal and adenoviral transfections were inefficient but activated the gene nonspecifically suggesting a potential of the gene to be activated endogenously.(5) Identification of the epitope for 7D5, a monoclonal antibody raised against flavocytochrome b5587D5 epitope was molecular-biologically located to the extracellular peptide conformation of gp91phox. The antibody is now undoubtedly available for diagnosis of X-CGD and monitoring the efficiency of the gene ttransfer to CGD patients.