Objectives To observe the expression of βig h3 in normal cornea and keratoconus and to elucidate the role of extracellular matrix in keratoconus Methods In situ hybridization was used to detect the expression of βig h3 in the cornea The cDNA library was screened with human βig h3 cDNA probe to locate βig h3 mRNA in cells Results Expression of βig h3 was found mainly in the stroma of the normal cornea and keratoconus, but decrease depending on the degree of keratopathy In some serious cases, no expression signal was detected The strongest expression was seen at the border of the normal region and keratoconus Conclusions βig h3,the structural component of the extracellular matrix, can affect cell adhensiveness in the development of corneal fibrous interstitial organization During the development of keratoconus, decreasing levels of βig h3 cause the diminution of corneal steadiness, which is related to formation of keratoconus