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A mouse model of classical late-infantile neuronal ceroid lipofuscinosis based on targeted disruption of the CLN2 gene results in a loss of tripeptidyl-peptidase I activity and progressive neurodegeneration.
Sleat DE, Wiseman JA, El-Banna M, Kim KH, Mao Q, Price S, Macauley SL, Sidman RL, Shen MM, Zhao Q, Passini MA, Davidson BL, Stewart GR, Lobel P. Sleat DE, et al. Among authors: wiseman ja. J Neurosci. 2004 Oct 13;24(41):9117-26. doi: 10.1523/JNEUROSCI.2729-04.2004. J Neurosci. 2004. PMID: 15483130 Free PMC article.