USHER SYNDROME AND ITS GENETIC CHARACTERIZATION

Usher syndrome is defined as an autosomal recessive disorder which caused by a mutation in any one of at least 10 genes resulting combination hearing loss and visual impairment. The defect the inner ear, whereas degeneration retinal cells called retinitis pigmentosa (RP), results vision. Based on severity age whensigns symptoms appear, there are three clinical subtypes (characterized I, II, III). People suffering from I deaf birth during first decade life, they begin to lose their Balance difficulties also exhibited them. Patients with II have but not birth. They vision later on. do show problems balance. having III gradually born deaf; may or In this review, those mouse models for usher will be discussed homologue humans was identified model called, “mouse model” based upon gene defects human beings.