Peroxisome-derived ether lipids regulate lysosomal exocytosis
Abstract
Lysosomes and peroxisomes are essential for cellular homeostasis, yet how their activities are coordinated remains poorly understood. Here, we identify peroxisome-derived ether lipids as key regulators of lysosomal function. A genome-wide CRISPR/Cas9 screen in LYSET-deficient mucolipidosis V cells revealed that disruption of ether lipid synthesis genes or peroxins markedly reduces lysosome accumulation and restores degradative capacity. Genetic or pharmacological inhibition of ether lipid synthesis enhanced lysosomal exocytosis and promoted the clearance of undigested material independently of mannose-6-phosphate trafficking. Conversely, supplementation with the ether lipid precursor hexadecylglycerol increased lysosome abundance, while reducing their degradative capacity. These findings uncover a peroxisome-lysosome metabolic axis, in which ether lipids act as bidirectional regulators of lysosomal number and function independently of the lysosomal master regulator TFEB. Our findings reveal how peroxisome-localized lipid metabolism modulates lysosomal homeostasis, and suggest potential new strategies to combat lysosomal and peroxisomal disorders.
Fields of Study & Subjects
Calcium signaling and nucleotide metabolism
Autophagy in Disease and Therapy
Lysosomal Storage Disorders Research
Publication details
- Journal / source
- The EMBO Journal
- Publisher
- Springer Science and Business Media LLC
- Volume
- 45
- Issue
- 11
- Pages
- 3699–3730
- Publication date
- 2 May 2026
- Language
- EN