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Enhanced Proteolytic Activities in Acral Peeling Skin Syndrome: A Role of Transglutaminase 5 in Epidermal Homeostasis Georgios Pampalakis, Dimitra Kiritsi, Eleni Zingkou, Claus-Werner Franzke, Manthoula Valari, Georgia Sotiropoulou Journal of Investigative Dermatology Volume 137, Issue 8, Pages (August 2017) DOI: /j.jid Copyright © 2017 The Authors Terms and Conditions
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Figure 1 Aberrantly increased epidermal proteolysis in acral peeling skin syndrome skin. In situ zymography with (a) BODIPY-casein, (b) gelatin, (c) and elastin substrate. The dermal-epidermal junction zone is indicated by a green dashed line. Higher epidermal proteolysis is associated with more pronounced desquamation (patients 686, 752 and 252). E indicates epidermis that is shown on the top. Scale bar: 200 μm. Journal of Investigative Dermatology , DOI: ( /j.jid ) Copyright © 2017 The Authors Terms and Conditions
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Figure 2 Expression of endogenous protease inhibitors in APSS skin. (a) Εndogenous inhibitors normally expressed in epidermis are deregulated in APSS. Protease inhibitor mRNA expression was quantified by RT-qPCR using 20 ng cDNA as template and two-step cycling conditions (95 °C for denaturation, 60 °C for annealing and extension, for 45 cycles). Gene-specific primers were designed or obtained from Qiagen (Hilden, Germany) (Supplementary Table S1 online). The identity of amplified PCR products was confirmed by DNA sequencing (VBC, Vienna, Austria). The control depicts the mean expression of four normal samples. (b) Abnormally high epidermal proteolysis is associated with enhanced degradation of desmoglein 1 (DSG1). DSG1 expression was analyzed by IHC using an anti-DSG1 antibody (H-290: sc-20114; Santa Cruz, Heidelberg, Germany) at dilution 1:300. Scale bar: 200 μm. Comparisons were made with the h score. The epidermis is shown on the top. The black dashed line indicates the dermis-epidermis junction. Representative image of one of three different controls is shown. APSS, acral peeling skin syndrome; IHC, immunohistochemistry; RT-qPCR, quantitative reverse transcription PCR. Journal of Investigative Dermatology , DOI: ( /j.jid ) Copyright © 2017 The Authors Terms and Conditions
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