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Endogenous β-glucocerebrosidase activity in Abca12(−/−)epidermis elevates ceramide levels after topical lipid application but does not restore barrier function

ABCA12 mutations disrupt the skin barrier and cause harlequin ichthyosis. We previously showed Abca12(−/−) skin has increased glucosylceramide (GlcCer) and correspondingly lower amounts of ceramide (Cer). To examine why loss of ABCA12 leads to accumulation of GlcCer, de novo sphingolipid synthesis w...

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Hlavní autoři: Haller, Jorge F., Cavallaro, Paul, Hernandez, Nicholas J., Dolat, Lee, Soscia, Stephanie J., Welti, Ruth, Grabowski, Gregory A., Fitzgerald, Michael L., Freeman, Mason W.
Médium: Artigo
Jazyk:Inglês
Vydáno: The American Society for Biochemistry and Molecular Biology 2014
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On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC3934733/
https://ncbi.nlm.nih.gov/pubmed/24293640
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1194/jlr.M044941
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