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Figure 2

Figure 2. The progression of AoV disease. From: Transcriptional regulation of heart valve development and disease.

(A) The normal aortic valve (AoV) is stratified into three highly specialized extracellular matrix (ECM) layers. The fibrosa layer is composed primarily of collagens, the spongiosa contains proteoglycans, and the ventricularis layer is rich in elastin. (B) Valvular interstitial cells (VIC) are activated during AoV disease, assuming a myofibroblast phenotype (red stars). (C) Valve sclerosis (orange) involves an overproduction of ECM molecules and maladaptive ECM remodeling, resulting in valve thickening. (D) Late stages of AoV disease are characterized by valve stenosis, which commonly involves valve calcification (black). Ao= aorta.

Elaine E. Wirrig, et al. Cardiovasc Pathol. ;20(3):162-167.
Figure 1

Figure 1. Diagram of embryonic valve development and valve maturation. From: Transcriptional regulation of heart valve development and disease.

(A) At mouse E10.5, immature cushions have formed in the atrioventricular (AV) canal and outflow tract (OFT). Endocardial derived cells populate the atrioventricular cushions (AVC) and portions of the OFT cushions (blue). Neural crest cells (NCC) (yellow) also contribute to the OFT cushions and aorticopulmonary septum (APS). (A’) Transcription factors including Twist1, Tbx20, Msx1, and Msx2 are important for AVC development. (B) At E13.5, the valve primordia (blue) begin remodeling and start to elongate. The dorsal mesenchymal protrusion (DMP) (purple) has fused with the AVC to form part of the atrial septum (AS). (B’) The transcription factors Sox9, Scleraxis, and Nfatc1 are important for valve remodeling. (C, D). The mature valves contain highly specialized extracellular matrix in three distinct layers, the collagenous fibrosa layer, the elastin-rich ventricularis/atrialis layers, and the proteoglycan abundant spongiosa layer. Ao= aorta, EC= endocardial cushions, EP= epicardium, EPDC= epicardial derived cells, IVS= interventricular septum, LA=left atrium, LV= left ventricle, RA= right atrium, RV= right ventricle.

Elaine E. Wirrig, et al. Cardiovasc Pathol. ;20(3):162-167.

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