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M.D. Associate Professor, Numune Training and Research Hospital, 3st Eye Clinic, Ankara/TURKEY Primary congenital glaucoma (PCG) is a particular type of glaucoma that is vary between societies. Although inherited autosomal recessive, isolated social life and consanguineous marriages affect the frequency. The major gene of the diseases is CYP1B1 (cytochrome p450 1 b1). However It is shown that the gene mutations MYOC (myocilin), and LTBP2 (latent-transforming growth factor beta-binding protein 2) may cause disease. While mutation screening of the existing genes is continuing, new researches goes on new candidate loci. The congenital glaucoma genetics will be discussed in detail in this article in the light of current knowledge. Keywords : Congenital glaucoma, genetic, mutation screening, CYP1B1, MYOC, LTBP2, FOXC1