The crystal structure of palmitoyl protein thioesterase 1 and the molecular basis of infantile neuronal ceroid lipofuscinosis

Mutations in palmitoyl-protein thioesterase 1 (PPT1), a lysosomal enzyme that removes fatty acyl groups from cysteine residues in modified proteins, cause the fatal inherited neurodegenerative disorder infantile neuronal ceroid lipofuscinosis. The accumulation of undigested substrates leads to the f...

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Detalhes bibliográficos

Publicado no:	Proc Natl Acad Sci U S A
Principais autores:	Bellizzi, John J., Widom, Joanne, Kemp, Christopher, Lu, Jui-Yun, Das, Amit K., Hofmann, Sandra L., Clardy, Jon
Formato:	Artigo
Idioma:	Inglês
Publicado em:	National Academy of Sciences 2000
Assuntos:	Biological Sciences
Acesso em linha:	https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC18274/ https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/10781062/ https://ncbi.nlm.nih.govhttps://doi.org/10.1073/pnas.080508097
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