Early Growth Response Protein 2 (EGR2)

The early growth response protein 2 is a transcription factor with three tandem C2H2-type zinc fingers. Mutations in this gene are associated with the autosomal dominant Charcot-Marie-Tooth disease, type 1. Using microarray expression profiling, Nagarajan et al. identified 98 known genes that were induced by Egr2 in Schwann cells. The putative Egr2 target genes included myelin proteins and enzymes required for synthesis of normal myelin lipids. Using RT-PCR to monitor Schwann cell gene expression, Nagarajan et al. confirmed that Egr2 is sufficient for induction of target genes including MPZ , PMP22 , MBP , MAG , CX32 , and periaxin. Using EGR2 DNA-binding domain mutants, Nagarajan et al.  demonstrated a dominant-negative inhibition of wildtype Egr2-mediated induction of essential myelin genes.