Abstract
Acid ceramidase is responsible for the ultimate step in the catabolism of (glyco)sphingolipids by hydrolysis of ceramide into sphingosine and free fatty acid. Deficiency in acid ceramidase is the molecular basis of Farber disease. Here we report the synthesis and characterization of an activity-based acid ceramidase probe.
MeSH terms
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Acid Ceramidase / analysis*
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Acid Ceramidase / metabolism*
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Ceramides / chemistry
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Ceramides / metabolism
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Enzyme Activation
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Fatty Acids / chemistry
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Fatty Acids / metabolism
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Hydrolysis
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Molecular Probes / chemical synthesis
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Molecular Probes / chemistry*
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Molecular Probes / metabolism*
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Molecular Structure
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Sphingolipids / chemistry
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Sphingolipids / metabolism
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Sphingosine / chemistry
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Sphingosine / metabolism
Substances
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Ceramides
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Fatty Acids
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Molecular Probes
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Sphingolipids
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Acid Ceramidase
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Sphingosine