Animal Models

As in humans, animals can also develop NCL. NCL that occurs naturally has been described in many species including dogs, cats, sheep, cattle, pigs, mice and birds. It is also possible to introduce genetic changes to create animals with NCL, especially mice and fish, but also larger animals such as pigs and sheep.

Some of these animals are being studied to understand more about disease progression, and are used in clinical trials to test new therapies. Others are used to identify new NCL genes that will help to complete the picture in terms of which genes cause NCL. Some genes that cause disease in animals may not have been found to cause disease in human patients.

DNA tests are available for these genes to identify carrier animals. Careful breeding prevents the disease being passed to any offspring.