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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Xehetasun bibliografikoak
Egile Nagusiak: Bellizzi, John J., Widom, Joanne, Kemp, Christopher, Lu, Jui-Yun, Das, Amit K., Hofmann, Sandra L., Clardy, Jon
Formatua: Artigo
Hizkuntza:Inglês
Argitaratua: The National Academy of Sciences 2000
Gaiak:
Biological Sciences
Sarrera elektronikoa:https://ncbi.nlm.nih.gov/pmc/articles/PMC18274/
https://ncbi.nlm.nih.gov/pubmed/10781062
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