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. 2014 Oct 16;24(5):525–544. doi: 10.1111/bpa.12181

Figure 7.

figure

An electron micrograph (EM) of a small dermal artery from a 28‐year‐old male CADASIL patient with p.Arg133Cys NOTCH3 mutation. Three small deposits of GOM (arrows) are detectable (already at this age) on a few vascular smooth muscle cells (VSMC). The subendothelial space is widened with accumulation of extracellular matrix proteins (asterisks). E = endothelium, N = nucleus (A). An EM of a dermal arteriole from a 19‐year‐old CADASIL patient [younger brother of the patient in (A), both sons of a male patient homozygous for p.Arg133Cys mutation] shows the paucity of GOMs (arrowhead) and the inset shows that true GOMs do occur already at this young age (B). A higher magnification EM of a dermal arteriole from an elderly patient at a more advanced stage of the disease. GOMs are present both in indentations on a VSMC (black asterisks) and in the intercellular space apparently disconnected to the VSMC (white asterisk) (C). Note the pinocytotic vesicles in the VSMC beneath the GOM (B and C insets). N = nucleus. [Fig. 7A is reproduced from article (62) with permission]