posterior polymorphous corneal dystrophy


posterior polymorphous corneal dystrophy

an autosomal dominant condition characterized by vesicular and linear abnormalities of the corneal endothelium; occasionally leads to corneal edema.

VSX1

A gene on chromosome 20p11.21 that encodes a homeobox protein which binds to the core of the locus control region of the red/green visual pigment gene cluster. It may regulate the activity of the locus control region (LCR) and the cone opsin genes at earlier stages of development.
Molecular pathology
VSX1 mutations can cause posterior polymorphous corneal dystrophy and keratoconus.

pos·te·ri·or pol·y·mor·phous cor·ne·al dys·tro·phy

(pos-tēr'ē-ŏr pol'ē-mōr'fŭs kōr'nē-ăl dis'trŏ-fē) An autosomal dominant condition characterized by vesicular and linear abnormalities of the corneal endothelium; occasionally leads to corneal edema