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Figure 6


Fig. 6. Co-expression of Adamts2 and Col3a1, and deficiency of procollagen III processing in Adamts2-/- mice. (A,B) Adamts2 is co-expressed with Col3a1 in the arterial wall, as shown at two developmental stages: 12.5 dpc carotid artery (A) and 17.5 dpc pulmonary artery (B). L, lung. (C) Expression of Col3a1 and Adamts2 in lung. Note the expression in lung mesnchyme but not in the epithelium of bronchial tubes (asterisk). (D) Co-expression of Adamts2 and Col3a1 in the mesenchyme of the palate (P). Adamts2 is also expressed in the perichondrium of the basisphenoid (BS), at which location Col3a1 is absent. (E) Co-expression of Adamts2 and Col3a1 in the peritoneum lining the liver (L, arrow), and mesentery of the gut (G) and pancreas (Pa). (F) Co-expression of Adamts2 and Col3a1 within the subepithelial layer of the developing urinary bladder. Scale bar: 100 µm. In D-F, Adamts2 expression is weaker than in lung and arteries, and Col3a1 expression is also significantly weaker and more dispersed. (G) Defective procollagen III processing in tissues from Adamts2-/- mice. Western blot analysis of procollagen isolated from mouse tissues with 0.15 M NaCl is shown. The tissue origin of the sample and the respective genotype is shown above each lane; procollagen III and collagen III molecular species are indicated on the left. Scanning densitometry of the different collagen III signals was used to calculate the proportion of fully processed molecules in the various tissues (lower panel). (H) Defective procollagen III processing in newly synthesized collagen in Adamts2-/- mice. Protein was extracted from sponge granulomas and western blotting used to define the molecular species of procollagen III that were present. Sponge granulomas from two Adamts2-/- mice and their wild-type littermates are shown. Scale bar: 100 µm.