| First Author | Elshatory Y | Year | 2003 |
| Journal | FEBS Lett | Volume | 538 |
| Issue | 1-3 | Pages | 207-12 |
| PubMed ID | 12633880 | Mgi Jnum | J:119180 |
| Mgi Id | MGI:3701409 | Doi | 10.1016/s0014-5793(03)00162-5 |
| Citation | Elshatory Y, et al. (2003) Early changes in gene expression in two models of Batten disease. FEBS Lett 538(1-3):207-12 |
| abstractText | Infantile and juvenile neuronal ceroid lipofuscinosis (NCLs) are progressive neurodegenerative disorders of childhood with distinct ages of clinical onset, but with a similar pathological outcome. Infantile and juvenile NCL are inherited in an autosomal recessive manner due to mutations in the CLN1 and CLN3 genes, respectively. Recently developed Cln1- and Cln3-knockout mouse models share similarities in pathology with the respective human disease. Using oligonucleotide arrays we identified reproducible changes in gene expression in the brains of both 10-week-old Cln1- and Cln3-knockout mice as compared to wild-type controls, and confirmed changes in levels of several of the cognate proteins by immunoblotting. Despite the similarities in pathology, the two mutations affect the expression of different, non-overlapping sets of genes. The possible significance of these changes and the pathological mechanisms underlying NCL diseases are discussed. |
