Juvenile neuronal ceroid lipofuscinoses (JNCL) is a lysosomal storage disorder associated with fatal neurodegeneration that is caused by mutations in CLN3. Most individuals with JNCL carry at least one allele with 1 kb deletion in CLN3 which results in the deletion of exons 7 and 8. There is a need ...
Discovery of proteins and pathways affected by overexpression of the protein TDP-43 in neurons cultured from wild-type and mice homogygous for the transgene.
Primary neuronal cultures were prepared from transgenic mice overexpressing wildtype human TDP-43 under the control of the prion promoter (hTDP-43Prp).
