Enzymes
UniProtKB help_outline | 2 proteins |
GO Molecular Function help_outline |
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Reaction participants Show >> << Hide
- Name help_outline 1,2,3-tri-(9Z,12Z)-octadecadienoylglycerol Identifier CHEBI:75844 (CAS: 537-40-6) help_outline Charge 0 Formula C57H98O6 InChIKeyhelp_outline HBOQXIRUPVQLKX-BBWANDEASA-N SMILEShelp_outline CCCCC\C=C/C\C=C/CCCCCCCC(=O)OCC(COC(=O)CCCCCCC\C=C/C\C=C/CCCCC)OC(=O)CCCCCCC\C=C/C\C=C/CCCCC 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 N-(30-hydroxytriacontanoyl)-sphing-4-enine Identifier CHEBI:138664 Charge 0 Formula C48H95NO4 InChIKeyhelp_outline KSVODRGOBYCXKF-HFBOQBPWSA-N SMILEShelp_outline C(CCCCCCCCCC)CC\C=C\[C@@H](O)[C@@H](NC(=O)CCCCCCCCCCCCCCCCCCCCCCCCCCCCCO)CO 2D coordinates Mol file for the small molecule Search links Involved in 2 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
- Name help_outline di-(9Z,12Z)-octadecadienoylglycerol Identifier CHEBI:138658 Charge 0 Formula C39H68O5 SMILEShelp_outline C(=C\C/C=C\CCCCC)\CCCCCCCC(=O)OCC(O*)CO* 2D coordinates Mol file for the small molecule Search links Involved in 9 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
- Name help_outline N-[30-(9Z,12Z-octadecadienoyloxy)-triacontanoyl]-sphing-4-enine Identifier CHEBI:34862 (CAS: 156170-27-3) help_outline Charge 0 Formula C66H125NO5 InChIKeyhelp_outline CJKGLEVYDCRGBX-FQYIUYQHSA-N SMILEShelp_outline [C@H]([C@@H](/C=C/CCCCCCCCCCCCC)O)(NC(=O)CCCCCCCCCCCCCCCCCCCCCCCCCCCCCOC(CCCCCCC/C=C\C/C=C\CCCCC)=O)CO 2D coordinates Mol file for the small molecule Search links Involved in 2 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
Cross-references
RHEA:55264 | RHEA:55265 | RHEA:55266 | RHEA:55267 | |
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Reaction direction help_outline | undefined | left-to-right | right-to-left | bidirectional |
UniProtKB help_outline |
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Gene Ontology help_outline | ||||
MetaCyc help_outline |
Related reactions help_outline
More general form(s) of this reaction
Publications
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PNPLA1 is a transacylase essential for the generation of the skin barrier lipid omega-O-acylceramide.
Ohno Y., Kamiyama N., Nakamichi S., Kihara A.
Lipids are the primary components of the skin permeability barrier, which is the body's most powerful defensive mechanism against pathogens. Acylceramide (ω-O-acylceramide) is a specialized lipid essential for skin barrier formation. Here, we identify PNPLA1 as the long-sought gene involved in the ... >> More
Lipids are the primary components of the skin permeability barrier, which is the body's most powerful defensive mechanism against pathogens. Acylceramide (ω-O-acylceramide) is a specialized lipid essential for skin barrier formation. Here, we identify PNPLA1 as the long-sought gene involved in the final step of acylceramide synthesis, esterification of ω-hydroxyceramide with linoleic acid, by cell-based assays. We show that increasing triglyceride levels by overproduction of the diacylglycerol acyltransferase DGAT2 stimulates acylceramide production, suggesting that triglyceride may act as a linoleic acid donor. Indeed, the in vitro analyses confirm that PNPLA1 catalyses acylceramide synthesis using triglyceride as a substrate. Mutant forms of PNPLA1 found in patients with ichthyosis exhibit reduced or no enzyme activity in either cell-based or in vitro assays. Altogether, our results indicate that PNPLA1 is directly involved in acylceramide synthesis as a transacylase, and provide important insights into the molecular mechanisms of skin barrier formation and of ichthyosis pathogenesis. << Less
Nat. Commun. 8:14610-14610(2017) [PubMed] [EuropePMC]
This publication is cited by 2 other entries.