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Objective  Gain-of-function (GOF) mutations in  RHBDF2  cause tylosis. Patients present with hyperproliferative skin, and keratinocytes from tylosis patients’ skin show an enhanced wound-healing phenotype. The curly bare mouse model of tylosis, carrying a GOF mutation in the  Rhbdf2  gene ( Rhbdf2    cub  ), presents with epidermal hyperplasia and shows accelerated cutaneous wound-healing phenotype through enhanced secretion of the epidermal growth factor receptor family ligand amphiregulin. Despite these advances in our understanding of tylosis, key questions remain. For instance, it is not known whether the disease is skin-specific, whether the immune system or the surrounding microenvironment plays a role, and whether mouse genetic background influences the hyperproliferative-skin and wound-healing phenotypes observed in  Rhbdf2    cub   mice.    Results  We performed bone marrow transfers and reciprocal skin transplants and found that bone marrow transfer from C57BL/6 (B6)- Rhbdf2    cub/cub   donor mice to B6 wildtype recipient mice failed to transfer the hyperproliferative-skin and wound-healing phenotypes in B6 mice. Furthermore, skin grafts from B6 mice to the dorsal skin of B6- Rhbdf2    cub/cub   mice maintained the phenotype of the donor mice. To test the influence of mouse genetic background, we backcrossed  Rhbdf2    cub   onto the MRL/MpJ strain and found that the hyperproliferative-skin and wound-healing phenotypes caused by the  Rhbdf2    cub   mutation persisted on the MRL/MpJ strain.

Tissue-specific role of RHBDF2 in cutaneous wound healing and hyperproliferative skin disease