Identified N- and C-termini which are not the result of trypsin digestion are indicated on the sequence of TGFBIp with the heterozygous A546D mutation indicated with a red D-letter above the sequence for the amyloid deposits (A) and periamyloid corneal tissue (B) from the LCD type 1 variant cornea. The positions of the cleavage sites in TGFBIp from the diseased cornea are compared with those found in the healthy cornea. Black and white arrows represent proteolytic cleavages identified only in the LCD type 1 variant corneal sample and healthy cornea, respectively. Gray arrows represent proteolytic cleavages identified in both the LCD type 1 variant corneal sample and healthy cornea. The underlined residue at each cleavage site indicates the terminus of the identified semitryptic peptide. The numbers above the arrows are the spectral counts of the peptide(s) with the proteolytic cleavage observed from the diseased cornea (upper number) and the healthy cornea (lower number). The colored bars below the sequences indicate the positions of the four FAS1 domains (FAS1–1: residues 103–235 (green); FAS1–2: residues 242–371 (red); FAS1–3: residues 376–498 (blue); and FAS1–4: residues 505–632 (yellow)). The cleavage sites detected only in TGFBIp from the healthy cornea are dispersed throughout the sequence. (A) Many of the cleavage sites in TGFBIp from the amyloid deposits are clustered in region F515-R533 positioned close to the mutated residue A546. (B) Half of the cleavage sites in TGFBIp from the periamyloid corneal sample are also located in the FAS1–4 domain in close proximity to the mutated residue A546. To access the data, click or select the words “Appendix 5.”