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Ubiquitous Transgene Expression of the Glucosylceramide-Synthesizing Enzyme Accelerates Glucosylceramide Accumulation and Storage Cells in a Gaucher Disease Mouse Model
Gaucher disease is a lysosomal storage disease caused by defective activity of acid β-glucosidase (GCase), which leads to the accumulation of its major substrates, glucosylceramide (GlcCer) and glucosylsphingosine (GlcSph) in many cells. To modulate cellular substrate concentration in viable mouse m...
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| Published in: | PLoS One |
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| Main Authors: | , , , , , , , |
| Format: | Artigo |
| Language: | Inglês |
| Published: |
Public Library of Science
2014
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| Subjects: | |
| Online Access: | https://ncbi.nlm.nih.gov/pmc/articles/PMC4281226/ https://ncbi.nlm.nih.gov/pubmed/25551612 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0116023 |
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