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Glycosylation, transport, and complex formation of palmitoyl protein thioesterase 1 (PPT1) – distinct characteristics in neurons

BACKGROUND: Neuronal ceroid lipofuscinoses (NCLs) are collectively the most common type of recessively inherited childhood encephalopathies. The most severe form of NCL, infantile neuronal ceroid lipofuscinosis (INCL), is caused by mutations in the CLN1 gene, resulting in a deficiency of the lysosom...

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Detalhes bibliográficos
Main Authors: Lyly, Annina, von Schantz, Carina, Salonen, Tarja, Kopra, Outi, Saarela, Jani, Jauhiainen, Matti, Kyttälä, Aija, Jalanko, Anu
Formato: Artigo
Idioma:Inglês
Publicado em: BioMed Central 2007
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC1906764/
https://ncbi.nlm.nih.gov/pubmed/17565660
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/1471-2121-8-22
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