Enzymes
| UniProtKB help_outline | 5 proteins |
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- Name help_outline a 3-acyl-sn-glycero-1-phospho-(1'-sn-glycerol) Identifier CHEBI:197425 Charge -1 Formula C7H13O9PR SMILEShelp_outline O(C[C@@H](COP(OC[C@H](CO)O)(=O)[O-])O)C(*)=O 2D coordinates Mol file for the small molecule Search links Involved in 6 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
- Name help_outline a 3-acyl-sn-glycero-1-phospho-(3ʼ-acyl-1ʼ-sn-glycerol) Identifier CHEBI:77717 Charge -1 Formula C8H12O10PR2 SMILEShelp_outline O(C[C@@H](COP(OC[C@H](COC(*)=O)O)(=O)[O-])O)C(*)=O 2D coordinates Mol file for the small molecule Search links Involved in 6 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
- Name help_outline sn-glycero-1-phospho-(1'-sn-glycerol) Identifier CHEBI:197411 Charge -1 Formula C6H14O8P InChIKeyhelp_outline LLCSXHMJULHSJN-WDSKDSINSA-M SMILEShelp_outline OC[C@@H](COP(OC[C@H](CO)O)(=O)[O-])O 2D coordinates Mol file for the small molecule Search links Involved in 5 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
Cross-references
| RHEA:77619 | RHEA:77620 | RHEA:77621 | RHEA:77622 | |
|---|---|---|---|---|
| Reaction direction help_outline | undefined | left-to-right | right-to-left | bidirectional |
| UniProtKB help_outline |
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Related reactions help_outline
Specific form(s) of this reaction
Publications
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The Batten disease gene product CLN5 is the lysosomal bis(monoacylglycero)phosphate synthase.
Medoh U.N., Hims A., Chen J.Y., Ghoochani A., Nyame K., Dong W., Abu-Remaileh M.
Lysosomes critically rely on bis(monoacylglycero)phosphate (BMP) to stimulate lipid catabolism, cholesterol homeostasis, and lysosomal function. Alterations in BMP levels in monogenic and complex neurodegeneration suggest an essential function in human health. However, the site and mechanism respo ... >> More
Lysosomes critically rely on bis(monoacylglycero)phosphate (BMP) to stimulate lipid catabolism, cholesterol homeostasis, and lysosomal function. Alterations in BMP levels in monogenic and complex neurodegeneration suggest an essential function in human health. However, the site and mechanism responsible for BMP synthesis have been subject to debate for decades. Here, we report that the Batten disease gene product CLN5 is the elusive BMP synthase (BMPS). BMPS-deficient cells exhibited a massive accumulation of the BMP synthesis precursor lysophosphatidylglycerol (LPG), depletion of BMP species, and dysfunctional lipid metabolism. Mechanistically, we found that BMPS mediated synthesis through an energy-independent base exchange reaction between two LPG molecules with increased activity on BMP-laden vesicles. Our study elucidates BMP biosynthesis and reveals an anabolic function of late endosomes/lysosomes. << Less
Science 381:1182-1189(2023) [PubMed] [EuropePMC]
This publication is cited by 4 other entries.