It is noteworthy that homozygous mutation of Cln3 or Cln6 in our cerebellar cell models of JNCL and vLINCL did not dramatically alter expression at the other NCL loci, at least by Affymetrix array analysis, supporting distinct primary functions for the differing NCL related proteins, consistent with the observation that NCL patients have varied storage material ultrastructure, age-at-onset, and order of symptom onset that typically correlates with the genetic etiology [13], [14]. This evidence concerns the gene NUCLEOLIN and neuronal ceroid lipofuscinosis.