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Animals can develop NCL. NCL that occurs naturally has been described in many species including dogs, cats, sheep, cattle, pigs, non-human primates, mice and birds.
It is possible to introduce genetic changes to create animals with NCL. This has been done in mice and fish especially, and also in larger animals such as pigs and sheep.
These animal models of specific NCL diseases are mostly being used to understand more about disease progression, and in preclinical studies to test the effectiveness of new therapies and safety of vectors before their use in clinical trials. Others may be used to understand the contribution of newly identified NCL genes to help complete the picture in terms of the genetic causes of NCL. Some genes found to cause disease in animals may not have been found to cause disease in human patients.
DNA tests may be available to identify carrier animals. Careful breeding can prevent the disease being passed to any offspring to reduce its occurence in that animal population.
A table that summarises what is known about animal models for which there is genetic information is provided here. We have not included case reports where disease has been described without genetic information, nor are non-mammalian models included here. The table includes details of two reviews which can provide further information about the models.
Table of animal NCL models
We welcome being provided with missing or new information to include in this table. This, and errors and omissions, should be sent to the Database Manager through the 'Contact Us' email.