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

Mutations in the CLN2 gene, which encodes a lysosomal serine protease, tripeptidyl-peptidase I (TPP I), result in an autosomal recessive neurodegenerative disease of children, classical late-infantile neuronal ceroid lipofuscinosis (cLINCL). cLINCL is inevitably fatal, and there currently exists no...

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Pubblicato in:J Neurosci
Autori principali: Sleat, David E., Wiseman, Jennifer A., El-Banna, Mukarram, Kim, Kwi-Hye, Mao, Qinwen, Price, Sandy, Macauley, Shannon L., Sidman, Richard L., Shen, Michael M., Zhao, Qi, Passini, Marco A., Davidson, Beverly L., Stewart, Gregory R., Lobel, Peter
Natura: Artigo
Lingua:Inglês
Pubblicazione: Society for Neuroscience 2004
Soggetti:
Neurobiology of Disease
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC6730049/
https://ncbi.nlm.nih.gov/pubmed/15483130
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1523/JNEUROSCI.2729-04.2004
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