Figure 4.

Recapitulation of NS Phenotype by ΔSPINK5 Keratinocytes In Vivo

Skin grafts were produced with healthy donor keratinocytes (HK Ctrl), NS patient keratinocytes (HKNS), ΔSPINK5 clone 1+4.2 (HKΔSPINK5), and immortalized ΔSPINK5 clone (HKΔSPINK5+E6E7). (A) Histological analysis (H&E staining) of grafts produced with HKΔSPINK5 and HKΔSPINK5+E6E7 shows the recapitulation of the typical hyperkeratotic phenotype of NS. (B) Human-specific involucrin immunostaining (hINV) confirms the human origin of the grafts. Insets depict the staining at the mouse-human skin border. (C–F) Immunoperoxidase detection of (C) LEKTI, (D) keratin K10, and (E) psoriasin, as well as (F) in situ zymography, reveals that the skin equivalents regenerated from the ΔSPINK5 keratinocytes fairly resemble those of NS. Scale bars, (A–E) 100 μm, (F) 50 μm.