PURPOSE. Keratoconus is a disease characterizedby thinning of the central and paracentral cornea and scarring in advancedcases. This study was performed to examine the expression of type XII collagen,proteins associated with hemidesmosomes, and β1 integrin in keratoconuscorneas. METHODS. Corneal buttons were collected from normal subjects and patientswith keratoconus and other corneal diseases. Immunofluorescence staining wasperformed on frozen sections for type XII collagen, bullous pemphigoid antigen(BP180), and integrin subunits α6, β4, and β1. RESULTS. To varying degrees, all proteins examined were expressed in normalhuman corneas. The staining intensity of type XII collagen was diminishedin keratoconus corneas in the epithelial basement membrane zone and the stromalmatrix. No significant variation was found in either the staining patternsor intensities for BP180, or integrins α6, β4, and β1. CONCLUSIONS. The level of type XII collagen was reduced in the epithelialbasement membrane zone and stromal matrices in keratoconus corneas. Thesealterations may affect critical interactions of the corneal epithelium withthe under-lying basement membrane, and cell-matrix interactions and matrixorganization in the stroma.