Systemic Administration of Tripeptidyl Peptidase I in a Mouse Model of Late Infantile Neuronal Ceroid Lipofuscinosis: Effect of Glycan Modification

Late-infantile neuronal ceroid lipofuscinosis (LINCL) is a recessive genetic disease of childhood caused by deficiencies in the lysosomal protease tripeptidyl peptidase I (TPP1). Disease is characterized by progressive and extensive neuronal death. One hurdle towards development of enzyme replacemen...

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Hlavní autoři: Meng, Yu, Sohar, Istvan, Wang, Lingling, Sleat, David E., Lobel, Peter
Médium: Artigo
Jazyk:Inglês
Vydáno: Public Library of Science 2012
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Research Article
On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC3391252/
https://ncbi.nlm.nih.gov/pubmed/22792360
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0040509
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