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Substrate Report for: Bis(monoacylglycerol)phosphate

Name Class
Bis(monoacylglycerol)phosphateTypeAcyl-Glycerol
Other_Name (11)
Chemical_Nomenclature[(2S)-2-hydroxy-3-[hydroxy-[(2S)-2-hydroxy-3-[(Z)-octadec-9-enoyl]oxypropoxy]phosphoryl]oxypropyl] (Z)-octadec-9-enoate
FormulaC42H79O10P
CAS_number1246303-12-7
MW792.07
Paper (5)
CommentBis(monoacylglycerol)phosphate (BMP) is a phospholipid crucial for lipid degradation and sorting in acidic organelles. Genetic and drug-induced lysosomal storage disorders (LSD) are associated with increased BMP concentrations in tissues and in the circulation BMP is found in intraluminal vesicles (ILVs) of late endosomes and lysosomes playing an important role for their structure and function. BMP is resistant to degradation by acid phospholipases and forms a docking station for lysosomal proteins. Lysosomal storage disorders (Niemann Pick disease, neuronal ceroid lipofuscinoses, Wolman disease) are associated with BMP accumulation in tissues. ABHD6 hydrolyzes BMP
Gene_locushuman-ABHD6
mouse-ABHD6
CID99647615
FamilyABHD6-Lip
InChIKeyGNCZTBXGLUHKAP-JOZUOZHYSA-N
CanonicalSMILESCCCCCCCCC=CCCCCCCCC(=O)OCC(COP(=O)(O)OCC(COC(=O)CCCCCCCC=CCCCCCCCC)O)O
InChIInChI=1S/C42H79O10P/c1-3-5-7-9-11-13-15-17-19-21-23-25-27-29-31-33-41(45)49-35-39(43)37-51-53(47,48)52-38-40(44)36-50-42(46)34-32-30-28-26-24-22-20-18-16-14-12-10-8-6-4-2/h17-20,39-40,43-44H,3-16,21-38H2,1-2H3,(H,47,48)/b19-17-,20-18-/t39-,40-/m0/s1

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Mail to: Nicolas Lenfant, Thierry Hotelier, Yves Bourne, Pascale Marchot and Arnaud Chatonnet.
Please cite: Lenfant 2013 Nucleic.Acids.Res. or Marchot Chatonnet 2012 Prot.Pept Lett.
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