Figure 3.

PreproEDN1 Domain Structure and Positions of Amino Acids Affected by Mutations

In the diagram at the top, bigEDN1 is shown in two shades of gray (the mature 21 amino acid peptide is the darker shade). Open arrowheads indicate furin cleavage sites. A filled arrowhead indicates the ECE cleavage site. Red arrows indicate the positions of substitutions identified in this report. The lower part of the figure is a multiple-sequence alignment of big endothelins and surrounding furin cleavage sites from selected species. Sequences used in the alignment were human EDN1 (RefSeq NP_001946.3), mouse EDN1 (RefSeq NP_034234.1), zebrafish Edn1 (RefSeq NP_571594.1), human EDN3 (RefSeq NP_000105.1), and mouse EDN3 (RefSeq NP_031929.1). The numbering above the alignment refers to the 21 amino acid mature peptide. Residues underlined with a red bar are affected by mutations in human EDN1 (this report), zebrafish edn1,¹¹ human EDN3,^1,22–25 and mouse Edn3^26,27 (see text for details). Black bars under the alignment indicate the two four-residue furin recognition sites. SP stands for signal peptide.