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Interactions of Sox10 and Egr2 in myelin gene regulation

  • Erin A. Jones (a1), Sung-Wook Jang (a1), Gennifer M. Mager (a2), Li-Wei Chang (a3), Rajini Srinivasan (a4), Nolan G. Gokey (a5), Rebecca M. Ward (a4), Rakesh Nagarajan (a3) and John Svaren (a4) (a6)...


Myelination in the PNS is accompanied by a large induction of the myelin protein zero (Mpz) gene to produce the most abundant component in peripheral myelin. Analyses of knockout mice have shown that the EGR2/Krox20 and SOX10 transcription factors are required for Mpz expression. Our recent work has shown that the dominant EGR2 mutations associated with human peripheral neuropathies cause disruption of EGR2/SOX10 synergy at specific sites, including a conserved enhancer element in the first intron of the Mpz gene. Further investigation of Egr2/Sox10 interactions reveals that activation of the Mpz intron element by Egr2 requires both Sox10-binding sites. In addition, both Egr1 and Egr3 cooperate with Sox10 to activate this element, which indicates that this capacity is conserved among Egr family members. Finally, a conserved composite structure of Egr2/Sox10-binding sites in the genes encoding Mpz, myelin-associated glycoprotein and myelin basic protein genes was used to screen for similar modules in other myelin genes, revealing a potential regulatory element in the periaxin gene. Overall, these results elucidate a working model for developmental regulation of Mpz expression, several facets of which extend to regulation of other peripheral myelin genes.


Corresponding author

Correspondence should be addressed to: John Svaren, 2015 Linden Drive, School of Veterinary Medicine, University of Wisconsin, Madison, WI 53706, USA phone: +1 608 263 4246 fax: +1 608 263 3926 email:



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