1. The Conundrum of Allogeneic Bone Marrow Transplantation for Epidermolysis Bullosa 
Jouni Uitto 
Journal of Investigative Dermatology 
Volume 138, Issue 5, Pages (May 2018)
DOI: /j.jid
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2. Figure 1
Conceptual illustration of bone marrow transplantation (BMT) for the treatment of epidermolysis bullosa (EB). BMT results in chimeric engraftment of donor-derived wild-type cells, including PDGFRα+/CXCR4+ mesenchymal stem cells (MSCs), into the recipient’s bone marrow. Necrotic skin in patients with EB releases cytokines and signaling molecules, such as high-mobility group box 1 (HMGB1) that recruits bone marrow-derived MSCs to circulation and subsequently homing to damaged skin through the stromal cell-derived factor-1α (SDF-1α)/CXCR4 axis. The damaged skin microenvironment creates a niche conducive for transdifferentiation of MSCs into keratinocytes (KCs) and fibroblasts (FBs) that elicit synthesis of proteins deficient in EB leading to tissue regeneration counteracting the disease.
(Adapted from Tamai and Uitto, 2016.)
Journal of Investigative Dermatology  , DOI: ( /j.jid )
Copyright © 2017 The Author Terms and Conditions