Disruption of PPT1 or PPT2 causes neuronal ceroid lipofuscinosis in knockout mice

PPT1 and PPT2 encode two lysosomal thioesterases that catalyze the hydrolysis of long chain fatty acyl CoAs. In addition to this function, PPT1 (palmitoyl-protein thioesterase 1) hydrolyzes fatty acids from modified cysteine residues in proteins that are undergoing degradation in the lysosome. PPT1...

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Bibliographische Detailangaben
Hauptverfasser: Gupta, Praveena, Soyombo, Abigail A., Atashband, Armita, Wisniewski, Krystyna E., Shelton, John M., Richardson, James A., Hammer, Robert E., Hofmann, Sandra L.
Format: Artigo
Sprache:Inglês
Veröffentlicht: The National Academy of Sciences 2001
Schlagworte:
Biological Sciences
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC61081/
https://ncbi.nlm.nih.gov/pubmed/11717424
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.251485198
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