Development of an acid ceramidase activity-based probe.
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.
Leiden Institute of Chemistry, Leiden University, P. O. Box 9502, 2300 RA Leiden, The Netherlands. h.s.overkleeft@chem.leidenuniv.nl j.m.f.g.aerts@lic.leidenuniv.nl.
Cécile M J
CM
Ouairy
Maria J
MJ
Ferraz
Rolf G
RG
Boot
Marc P
MP
Baggelaar
Mario
M
van der Stelt
Monique
M
Appelman
Gijsbert A
GA
van der Marel
Bogdan I
BI
Florea
Johannes M F G
JM
Aerts
Herman S
HS
Overkleeft
D055573:Acid Ceramidase / Q000032:analysis* / Q000378:metabolism*; D002518:Ceramides / Q000737:chemistry / Q000378:metabolism; D004789:Enzyme Activation; D005227:Fatty Acids / Q000737:chemistry / Q000378:metabolism; D006868:Hydrolysis; D015335:Molecular Probes / Q000138:chemical synthesis / Q000737:chemistry* / Q000378:metabolism*; D015394:Molecular Structure; D013107:Sphingolipids / Q000737:chemistry / Q000378:metabolism; D013110:Sphingosine / Q000737:chemistry / Q000378:metabolism
D002518:Ceramides; D005227:Fatty Acids; D015335:Molecular Probes; D013107:Sphingolipids; D055573:Acid Ceramidase; D013110:Sphingosine