PR002659 (Project)

Description:Lysosomes play a central role in cellular homeostasis by degrading proteins internalized through endocytosis and autophagy and recycling their components for organelle biogenesis. Lysosomal Storage Disorders (LSDs) represent a diverse group of diseases that disrupt lysosomal degradation, ion transport, and lipid metabolism. Among these, sphingolipidoses involve defects in glycosphingolipid breakdown, with gene products such as GBA1 identified, and others like SMPD1 and ASAH1 proposed, as genetic risk factors for Parkinson’s disease, although the underlying mechanisms remain poorly defined. In this dataset, we profile lipids from wildtype and ASAH1-/- HeLa cells, as well as from lysosomes isolated from these cells using LysoIP. Consistent with the loss of ASAH1 function, we observe elevated ceramide levels in knockout cells.