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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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Библиографические подробности
Опубликовано в: :Biochem J
Главные авторы: Kim, Kwi-Hye, Pham, Christine T., Sleat, David E., Lobel, Peter
Формат: Artigo
Язык:Inglês
Опубликовано: 2008
Предметы:
Online-ссылка: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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