A STUDY OF PAKISTANI FAMILIES WITH LEBER'S CONGENITAL AMAUROSIS

Abstract

Eye is one of the five main sense organs of human. Three layers of tissue enclose the eye (Figure 1.1). Sclera and cornea compose the outer layer, whereas iris, ciliary body and choroid make the middle layer (Benedek, 1971). But innermost layer constitutes of retina which contains the photoreceptors. It acts as a light-sensitive layer in eye and covers 65% of its inner surface, measuring around 42mm in diameter. It is divided into two distinctive areas, an outer retinal pigment epithelium (RPE) and inner sensorineural retina (Koenekoop, 2004). The retina is a highly specialized tissue composed of six main cell types, namely the cone and rod photoreceptors, bipolar, horizontal, amacrine, MUlier and ganglion cells (Figure 1.2).