Heterogeneity in Keratoconus: Possible Biochemical Basis

Total protein and collagen content in normal and keratoconus corneas were determined. The protein content (expressed as a function of dry weight) in all keratoconus corneal samples was lower than that found in normal corneas. However, among the 11 keratoconus corneas examined, only 7 (group A) had the same hydroxyproline content (expressed as a function of dry weight) as normal corneas; 4 others (group B) showed significantly less. In tissue culture, four strains derived from keratoconus stroma (group I) produced total protein at the same rate as cells from normal controls. Four other strains (group II), however, had a decreased rate of protein synthesis. The amount of collagenous protein synthesized per microgram DNA by group I strains was similar to that found in normal cultures, whereas it was significantly reduced in group II cultures. We suggest that group I strains represent group A corneas. Group II strains, with a reduced level of both protein and collagen synthesis, may represent group B corneas. The defect in this group appears to be decreased total synthetic activity of corneal cells. The variation in our results suggests that keratoconus is a heterogeneous disease. The heterogeneity may explain the contradictory data that exist in the literature.