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Manon C. Zweers, Ivonne M. van Vlijmen-Willems, Toin H

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1 Deficiency of Tenascin-X Causes Abnormalities in Dermal Elastic Fiber Morphology 
Manon C. Zweers, Ivonne M. van Vlijmen-Willems, Toin H. van Kuppevelt, Robert P. Mecham, Peter M. Steijlen, Jim Bristow, Joost Schalkwijk  Journal of Investigative Dermatology  Volume 122, Issue 4, Pages (April 2004) DOI: /j X x Copyright © 2004 The Society for Investigative Dermatology, Inc Terms and Conditions

2 Figure 1 van Gieson's staining of elastic fibers in human skin biopsies. In normal non-sun exposed skin, Elastin-van Gieson's staining shows a typical candelabra-like pattern oriented perpendicular towards the basal lamina (A). In TNX-deficient patients (B, C), the candelabra-like structure is grossly disturbed. Note the short, sparsely branched structures in b and the horizontally oriented, coarse fragments and in c fragmentation of elastic fibers in TNX-deficient skin. Morphometric analysis of five patients and eight controls showed a significant reduction in length of the elastic fibers (p<0.01) (D), number of branches per elastic fiber (p<0.01) (E) and relative collagen density (p<0.05) (F) in TNX-deficient dermis. Asterisks denote values significantly different from control (*p<0.05, **>p<0.01). Scale bar=50 μm. Journal of Investigative Dermatology  , DOI: ( /j X x) Copyright © 2004 The Society for Investigative Dermatology, Inc Terms and Conditions

3 Figure 2 Safranin-O histochemistry and decorin immunostaining. Safranin-O staining shows a similar glycosaminoglycan distribution in control (A) and TNX-deficient skin (B). Decorin is equally present in control (C) and TNX-deficient skin (D). TNX is distributed throughout the entire dermis of control skin (E) and is absent from TNX-deficient skin (F). Scale bar=25 μm for A–D, 50 μm for E and F. Journal of Investigative Dermatology  , DOI: ( /j X x) Copyright © 2004 The Society for Investigative Dermatology, Inc Terms and Conditions

4 Figure 3 Immunohistochemical staining for elastin and fibrillin-1. Note the typical structure of thin, ascending elastin-positive fibers in the papillary dermis (A) and the thicker fibers in the reticular dermis of normal skin (C). Fibrillin-1 staining shows a similar pattern (E). In the papillary dermis of the TNX-deficient skin, elastin-positive and fibrillin-positive fibers are sparse and fragmented (B) and (F). In the reticular dermis, clumping and fragmentation of elastin-positive structures is observed in TNX-deficient patients (D). Scale bar=25 μm. Journal of Investigative Dermatology  , DOI: ( /j X x) Copyright © 2004 The Society for Investigative Dermatology, Inc Terms and Conditions

5 Figure 4 Ultrastructural analysis of elastic fibers in dermis. TNX-deficient skin (A–E) shows many abnormalities in elastic fibers. (A) Fragmented appearing elastic fiber. (B) The elastin matrix appears to be deposited independent of the microfibrillar scaffold. Note the absence of microfibrils within the amorphous elastin and the holes containing microfibrillar material inside the elastic fiber. (C) Note the blebs of amorphous elastin without microfibrils as indicated by arrows. (D) Excessive amounts of elastin containing electron-dense debris. (E) Microfibrils are excessive and appear to be tangled in TNX-deficient patients. (F) Elastic fiber in skin from age-matched control. Arrows: abnormalities of elastic fibers; arrowheads: debris of microfibrils; asterisk: normal appearing elastic fiber. Scale bar; A–D, F=0.4 μm, E=0.17 μm. Journal of Investigative Dermatology  , DOI: ( /j X x) Copyright © 2004 The Society for Investigative Dermatology, Inc Terms and Conditions

6 Figure 5 Fibrillin-1 and TNX deposition and secretion. Normal fibroblasts secrete TNX into the medium in contrast to TNX-deficient fibroblasts, as shown by western blotting (A). No difference is seen in fibrillin-1 secretion into the medium (B), or in deposition of fibrillin-1 in the extracellular matrix by control fibroblasts (C) and TNX-deficient fibroblasts (D). Staining of TNX shows intracellular localization in normal fibroblasts (E) and absence of staining in TNX-deficient fibroblasts (F). Scale bar=25 μm. Journal of Investigative Dermatology  , DOI: ( /j X x) Copyright © 2004 The Society for Investigative Dermatology, Inc Terms and Conditions

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