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- W2025306989 abstract "Loss of function of galactosylceramidase lysosomal activity causes demyelination and vulnerability of various neuronal populations in Krabbe disease. Psychosine, a lipid-raft-associated sphingolipid that accumulates in this disease, is thought to trigger these abnormalities. Myelin-free <i>in vitro</i> analyses showed that psychosine inhibited fast axonal transport through the activation of axonal PP1 and GSK3β in the axon. Abnormal levels of activated GSK3β and abnormally phosphorylated kinesin light chains were found in nerve samples from a mouse model of Krabbe disease. Administration of GSK3β inhibitors significantly ameliorated transport defects <i>in vitro</i> and <i>in vivo</i> in peripheral axons of the mutant mouse. This study identifies psychosine as a pathogenic sphingolipid able to block fast axonal transport and is the first to provide a molecular mechanism underlying dying-back degeneration in this genetic leukodystrophy." @default.
- W2025306989 created "2016-06-24" @default.
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- W2025306989 date "2013-06-12" @default.
- W2025306989 modified "2023-10-11" @default.
- W2025306989 title "The Sphingolipid Psychosine Inhibits Fast Axonal Transport in Krabbe Disease by Activation of GSK3 and Deregulation of Molecular Motors" @default.
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- W2025306989 doi "https://doi.org/10.1523/jneurosci.0217-13.2013" @default.
- W2025306989 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3682375" @default.
- W2025306989 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/23761900" @default.
- W2025306989 hasPublicationYear "2013" @default.
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