CHST6 is mainly expressed by the keratocytes both in vivo and in vitro.70 Defects in this gene are associated with macular corneal dystrophy (MCD), a rare autosomal recessive inherited disorder characterized by abnormal deposits of unsulfated KSPGs that causes progressive stromal haziness leading to visual impairment.71,72. Here, CHST6 is linked to autosomal recessive disease.