One of the still unanswered questions of juvenile NCL is what function, if any, the affected protein (CLN3) has in the cell. This and other not as yet clarified research areas will hopefully be elucidated by selectively awarding and arranging start up funding.    

 

Basic research can simulate the course of the disease by means of several model systems. Possible model systems include fruit flies, which have a very well characterised nervous system. Baker's yeast also has a CLN3-related gene and is used mainly by Prof. Pearce's group. Appropriate mouse models have been studied more closely in the EU project "CNL Models".

 

Using these research findings, new active substances need to be found that prevent continuous destruction of sensitive nerve cells.