Substrate Report for: Bis(monoacylglycerol)phosphate
Bis(monoacylglycerol)phosphate | Type | Acyl-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 |
| Formula | C42H79O10P |
| CAS_number | 1246303-12-7 |
| MW | 792.07 |
| |
| Paper (5) |
| Comment | Bis(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_locus | human-ABHD6 |
| | mouse-ABHD6 |
| CID | 99647615 |
| Family | ABHD6-Lip |
| InChIKey | GNCZTBXGLUHKAP-JOZUOZHYSA-N |
| CanonicalSMILES | CCCCCCCCC=CCCCCCCCC(=O)OCC(COP(=O)(O)OCC(COC(=O)CCCCCCCC=CCCCCCCCC)O)O |
| InChI | InChI=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 |
|
|