Figure 2.

Plectin protein expression in keratinocytes. a: Immunofluorescence microscopy. Sections of skin biopsies of patient II-1 were stained with mAb 5B3 and anti-mouse FITC-conjugated IgG. Loss of staining in patient’s skin (P) compared to sections of normal skin (C) was observed. In patient’s skin, the position of a blister roof and blister floor at the dermo-epidermal junction is indicated by arrowheads, the cutaneous basement membrane is marked by arrows. The apparent staining at the top of the epidermis in patient’s skin is caused by autofluorescence of the stratum corneum. Original magnification, ×1:20. b: Western blot analysis. Skin keratinocytes from patient II-1 were cultured and protein extracts were analyzed using mAb 5B3. A grossly reduced full-length protein was detected in patient keratinocytes (P) as compared to controls (C). The positions of molecular weight markers are indicated.