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. 2013 Jul 16;288(35):25109–25118. doi: 10.1074/jbc.M113.477695

FIGURE 1.

FIGURE 1.

Structural model of keratoepithelin. The image shows a simplified diagram of keratoepithelin, which comprises cysteine-rich domain (EMI), four fasciclin 1 domains (FAS1), and an Arg-Gly-Asp (RGD) motif. Arg-124 and Arg-555 residues are hot mutations related to some corneal dystrophies. The crystal structure shows a FAS1–4 domain from human keratoepithelin (PDB ID: 2vxp). The synthesized peptides derived from the 110–131 residues of the FAS1–1 domain contain the Arg-124 residue. R124H (H-type) and R124C (C-type) mutations cause Avellino and classic lattice corneal dystrophy, respectively. R-type represents the wild-type.