Dipeptidyl-peptidase I does not functionally compensate for the loss of tripeptidyl-peptidase I in the neurodegenerative disease late-infantile neuronal ceroid lipofuscinosis

LINCL (late-infantile neuronal ceroid lipofuscinosis) is a fatal neurodegenerative disease resulting from mutations in the gene encoding the lysosomal protease TPPI (tripeptidyl-peptidase I). TPPI is expressed ubiquitously throughout the body but disease appears restricted to the brain. One explanat...

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Publicado en:Biochem J
Main Authors: Kim, Kwi-Hye, Pham, Christine T., Sleat, David E., Lobel, Peter
Formato: Artigo
Idioma:Inglês
Publicado: 2008
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Article
Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC4353488/
https://ncbi.nlm.nih.gov/pubmed/18570628
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1042/BJ20080411
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