| First Author | Hünefeld C | Year | 2018 |
| Journal | J Invest Dermatol | Volume | 138 |
| Issue | 5 | Pages | 1157-1165 |
| PubMed ID | 29203359 | Mgi Jnum | J:261186 |
| Mgi Id | MGI:6154402 | Doi | 10.1016/j.jid.2017.10.035 |
| Citation | Hunefeld C, et al. (2018) Bone Marrow-Derived Stem Cells Migrate into Intraepidermal Skin Defects of a Desmoglein-3 Knockout Mouse Model but Preserve their Mesodermal Differentiation. J Invest Dermatol 138(5):1157-1165 |
| abstractText | Inherited forms of epidermolysis bullosa are blistering diseases of the skin and mucosa resulting from various gene mutations. Transplantation of bone marrow-derived stem cells might be a promising systemic treatment for severe dystrophic or junctional epidermolysis bullosa, but many key questions remain unresolved. Two open questions of clinical interest are whether systemically transplanted bone marrow-derived stem cells of mesodermal origin might be able to transdifferentiate into keratinocytes with an ectodermal phenotype and whether these cells are also capable of repairing a specific intraepidermal gene defect. To address these questions, we transplanted bone marrow-derived stem cells into mice with a blistering disease exclusively localized to the epidermis resulting from a functional knockout of desmoglein-3 (Dsg3). We found that Dsg3(+) donor-derived cells migrate into the recipient epidermis. However, these cells failed to restore the missing Dsg3 mRNA and DSG3 protein expression in the transplanted Dsg3(-/-) mice. The donor-derived cells found in the epidermis preserved their CD45(+) hematopoietic origin, and no transdifferentiation into integrin alpha6(+) keratinocytes or integrin alpha6(+)/CD34(+) epidermal stem cells occurred. Our results indicate that bone marrow-derived stem cells preserve their mesodermal fate after systemic transplantation and are not capable of treating patients with epidermolysis bullosa with an intraepidermal skin defect. |
