Expression of mRNA encoding the secondary granule protein hCAP18 and primary granule protein BPI is severely reduced in the PBMCs of the SGD patient.

(A) RT-PCR analysis of total RNA prepared from the PBMCs of the patient and father. The cDNAs were analyzed for expression of the primary granule genes MPO, HNP-1 and HNP-3 (HNP1/3), and BPI; the secondary granule geneslactoferrin (LF) and hCAP18; and the control 18S rRNA. The products were Southern blotted and hybridized with either internal oligonucleotide or cDNA probes. (B) Induced expression of C/EBPε₃₂ in U937 activates hCAP18 expression. U937 cells stably transformed with a zinc-inducible empty vector (pMT) or one containing an insert for C/EBPε₃₂ (pMT-ε₃₂) were treated either without (−) or with (+) 100 μM ZnSO₄for 24 hours. RNA was harvested and analyzed by Northern blot hybridization with a probe for hCAP18. The blot was stripped and subsequently hybridized for β-actin.