Experimental Models
Many different small animal models are used for NCL research. These range from single celled organisms, such as yeasts, through nematode worms or flies,
which have simple nervous systems, through to vertebrate fish models.
Each model has its own advantages. These include access to the complete sequence of their genome, their genetic tractability and easily monitored brain development.
The use of some of these in NCL research is still under development
List of Models
Budding Yeast - Saccharomyces cerevisiae
- BTN1 - used as a model for CLN3 or JNCL
Fission Yeast - Schizosaccharonmyces pombe
- btn1 - used as a model for CLN3 or JNCL
- ppt1 - used as a model for CLN1 or INCL
Nematotode worm - Caenorhabditis elegans
- cln-1 - used as a model for CLN1 or INCL
- cln-3 - used as a model for CLN3 or JNCL
Fly - Drosophila melanogaster
- cln-1 - used as a model for CLN1 or INCL
- cln-3 - under development
- ctsd - used as a model for CTSD/CONCL, the Swedish landrace sheep
Zebrafish
- cln1 - under development
- cln2 - under development
- cln3 - under development
- cln5 - under development
- cln6 - under development
- cln8 - under development
- ctsd - under development