Human iPSC-derived myelinating organoids and globoid cells to study Krabbe disease
Human iPSC-derived myelinating organoids and globoid cells to study Krabbe disease
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Publisher
United States: Public Library of Science
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Language
English
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Publisher
United States: Public Library of Science
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Scope and Contents
Contents
Krabbe disease (Kd) is a lysosomal storage disorder (LSD) caused by the deficiency of the lysosomal galactosylceramidase (GALC) which cleaves the myelin enriched lipid galactosylceramide (GalCer). Accumulated GalCer is catabolized into the cytotoxic lipid psychosine that causes myelinating cells death and demyelination which recruits microglia/macr...
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Full title
Human iPSC-derived myelinating organoids and globoid cells to study Krabbe disease
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TN_cdi_plos_journals_3141380343
Permalink
https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_plos_journals_3141380343
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ISSN
1932-6203
E-ISSN
1932-6203
DOI
10.1371/journal.pone.0314858
