AIM:To investigate the expression of visual system homeobox 1(VSX1)and myofibroblast marker alpha smooth muscle actin(α-SMA)in keratoconus(KC).METHODS:Thirty corneal tissue were collected from KC patients after corneal transplantation and 15 normal donor corneas were obtained.All corneal tissues divided into 4 parts for different detections.Scanning electron microscopy was used to observe the ultrastructure of the specimens.VSX1 andα-SMA localization in cornea tissues was detected using immunofluorescence histochemistry.Reverse transcription-quantitative polymerase chain reaction(RT-qPCR)and Western blot were performed to analyze the expression level of VSX1 andα-SMA.RESULTS:Compared to normal cornea tissue,the collagen fibers in KC stroma were distortional and attenuated and keratocytes were abnormally changed.VSX1 andα-SMA located in the corneal stroma.The mRNA and protein expression level of VSX1 in KC were about 3 times as high as that of normal tissue(P<0.001).α-SMA was hardly expressed in the normal corneas,however,its expression in the KC was about 1.5 times higher than that of the normal corneas(P<0.0001).