Peroxisome-derived ether lipids regulate lysosomal exocytosis

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.

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Publication Details

Journal
The EMBO Journal
Published
2026-05-02
DOI
https://doi.org/10.1038/s44318-026-00791-3
Primary Topic
Calcium signaling and nucleotide metabolism
Type
article
Field-Weighted Citation Impact
0.00

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article

Peroxisome-derived ether lipids regulate lysosomal exocytosis

Gonçalo Vale, Jeffrey G. McDonald, Aleksander Wielenga, Yamuna Krishnan et al.
The EMBO Journal
Calcium signaling and nucleotide metabolism
article

Peroxisome-derived ether lipids regulate lysosomal exocytosis

Gonçalo Vale, Jeffrey G. McDonald, Aleksander Wielenga, Yamuna Krishnan, D Henn, Junyi Zou, Jiaxuan Liang, Zhengjun Dong, Bala Burugula, Ming Li, Liang Chen, Jacob Kitzman
article en

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.

The EMBO Journal
University of Michigan (US), University of Chicago (US), Michigan Medicine (US), The University of Texas Southwestern Medical Center (US)
National Institute of General Medical Sciences, National Center for Advancing Translational Sciences, Eunice Kennedy Shriver National Institute of Child Health and Human Development, NHLBI Division of Intramural Research, Division of Diabetes, Endocrinology, and Metabolic Diseases
Openalex Percentile: Top 13%
Calcium signaling and nucleotide metabolism
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