{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Tsai YT"],"funding":["NCI NIH HHS","NIGMS NIH HHS"],"pagination":["45-58.e8"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC12818558"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["33(1)"],"pubmed_abstract":["1-Deoxysphingolipids are non-canonical sphingolipids linked to several diseases, yet their cellular effects are poorly understood. Here, we utilize lipid chemical biology approaches to investigate the role of 1-deoxysphingolipid metabolism on the properties and functions of secretory membranes. We applied organelle-specific bioorthogonal labeling to visualize the subcellular distribution of metabolically tagged sphingolipids. We observed that 1-deoxysphingolipids are retained in the endoplasmic reticulum (ER) and specifically in ER exit sites (ERESs), suggesting that they do not efficiently sort into vesicular carriers. Cell lines expressing disease-associated variants of serine palmitoyl-CoA transferase accumulated 1-deoxysphingolipids, which were accompanied by a reduction in ER membrane"],"journal":["Cell chemical biology"],"pubmed_title":["1-deoxysphingolipids dysregulate membrane properties and cargo trafficking in the early secretory pathway."],"pmcid":["PMC12818558"],"funding_grant_id":["R35 GM133565","T32 GM139795","R01 CA234245","R35 GM142960","T32 GM146648","R35 GM141939"],"pubmed_authors":["McGregor GH","Neal S","Laguerre A","Metallo CM","Budin I","Kuerschner L","Solorio-Kirpichyan K","Brea RJ","Seidel O","Devaraj NK","Varma R","Tsai YT","Cordes T","Wong AM","Lipp NF"],"additional_accession":[]},"is_claimable":false,"name":"1-deoxysphingolipids dysregulate membrane properties and cargo trafficking in the early secretory pathway.","description":"1-Deoxysphingolipids are non-canonical sphingolipids linked to several diseases, yet their cellular effects are poorly understood. Here, we utilize lipid chemical biology approaches to investigate the role of 1-deoxysphingolipid metabolism on the properties and functions of secretory membranes. We applied organelle-specific bioorthogonal labeling to visualize the subcellular distribution of metabolically tagged sphingolipids. We observed that 1-deoxysphingolipids are retained in the endoplasmic reticulum (ER) and specifically in ER exit sites (ERESs), suggesting that they do not efficiently sort into vesicular carriers. Cell lines expressing disease-associated variants of serine palmitoyl-CoA transferase accumulated 1-deoxysphingolipids, which were accompanied by a reduction in ER membrane","dates":{"release":"2026-01-01T00:00:00Z","publication":"2026 Jan","modification":"2026-06-06T19:32:50.076Z","creation":"2026-06-04T03:11:57.17Z"},"accession":"S-EPMC12818558","cross_references":{"pubmed":["41544616"],"doi":["10.1016/j.chembiol.2025.12.006"]}}