The family of inherited ocular diseases that is collectively known as retin
itis pigmentosa is a major cause of progressive retinal disease worldwide.
As such, this family of diseases has been the object of much scientific scr
utiny, both clinical and basic. The recent application of molecular genetic
analyses has heralded the rapid elucidation of the underlying gene defects
in many cases. In this article, the fundamental clinical and electroretino
graphic characteristics of retinitis pigmentosa will be recalled. Additiona
lly, the current understanding of the genetic causes of retinitis pigmentos
a will be reviewed, and the identified causative genes will be classified i
nto groups related by function.