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. 2017 Jan 5;100(2):216–227. doi: 10.1016/j.ajhg.2016.12.010

Figure 5.

Figure 5

Ultrastructural Studies of the ECM

TEM of the dermis of PI:1 and PIII:1 showed collagen abnormalities with increased interfibrillar space and more variable fibril diameters than did a control sample (top row; scale bars represent 250 nm). Elastic fibers appeared normal in PIII:1 but were very irregular, fragmented, and almost absent in PI:1 (bottom row; scale bars represent 500 nm).