Micro Array Project on CLN3 Mouse

by 

Dr. David Pearce

"Here at the Center for Aging and Developmental Biology, University of Rochester School of Medicine we are applying this technology into the understanding of the devastating children's disorder, Batten disease. Batten disease results from lacking one particular component or protein called CLN3. We are therefore interested in seeing what happens to all the other components or proteins when one of these, CLN3, is lacking. This is very important as Batten disease is a progressive neurodegenerative disease. By revealing which other proteins have different "expression" in a normal situation as compared to lacking CLN3, we will gain valuable insight into how cells respond to the absence of one of these components. Furthermore, we will know a great deal more about the biology of the CLN3 component that when missing results in this terrible disease. This will lead to a greater understanding of the disease. Once we know how the other 79,999 components of a cell respond to the lack of CLN3, we essentially have a foot-print for the response to lacking this component. Ultimately this foot-print may be used to assess the ability to compensate a lack of CLN3 with a therapeutic strategy".

"Using the Affymetrix gene chip system we are comparing gene expression between a normal mouse and a mouse lacking CLN3, the gene that is associated to Batten disease.

Specifically this system currently has 19,000 murine genes or EST’s arrayed onto a chip in the form of olignucleotides. We can therefore probe this array to monitor expression levels of genes from any given situation. Initially we are extracting mRNA from the retina and cerebellum, two tissues known to be affected severely in Batten disease, with the purpose of comparing gene expression in these tissues. We hypothesize that the cln3 defect will manifest altered gene expression as compared to normal, and reveal valuable information as to the molecular basis of Batten disease".

For more information on this project please contact:

Dr. David Pearce University of Rochester School of Medicine