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The lamin B receptor (LBR) is an integral nuclear envelope protein that interacts with chromatin and has homology to sterol reductases. Mutations inLBRresult in Pelger–Huët anomaly and HEM–Greenberg skeletal dysplasia, whereas in miceLbrmutations result in ichthyosis. To further understand the function of the LBR and its role in disease, we derived a novel mouse model with a gene-trap insertion into theLbrlocus (Lbr   GT/GT). Phenotypically, theLbr   GT/GTmice are similar to ichthyosis mice. TheLbr   GT/GTgranulocytes lack a mature segmented nucleus and have a block in late maturation. Despite these changes in nuclear morphology, the innate granulocyte immune function in the killing ofStaphylococcus aureusbacteria appears to be intact. Granulocyte differentiation requires the transcription factor C/EBPε. We identified C/EBPε binding sites within theLbrpromoter and used EMSAs and luciferase assays to show thatLbris transcriptionally regulated by C/EBPε. Our findings indicate that theLbr   GT/GTmice are a model for Pelger–Huët anomaly and thatLbr, under transcriptional regulation of C/EBPε, is necessary for morphological but not necessarily functional granulocyte maturation.

The lamin B receptor under transcriptional control of C/EBPε is required for morphological but not functional maturation of neutrophils