ArticleThe Journal of cell biology2016
MICOS and phospholipid transfer by Ups2-Mdm35 organize membrane lipid synthesis in mitochondria.
Article in The Journal of cell biology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 85 papers.
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Who cites it
85 citing papers in PubMed, 208 citations in OpenAlex.
- Rewiring mitochondrial phosphatidylethanolamine metabolism identifies new and unaccounted trafficking steps.Journal of lipid research · 2026Article
- MICOS and MIMAS, multifunctional assemblies linking mitochondrial biogenesis, architecture, and function.Protein science : a publication of the Protein Society · 2026Review
- Rewiring Mitochondrial Phosphatidylethanolamine Metabolism Identifies New and Unaccounted Trafficking Steps.bioRxiv : the preprint server for biology · 2026Article
- SLMO2 inhibits apoptosis in ovarian cancer cells by modulating mitochondrial function via TRIAP1.Histology and histopathology · 2026Article
- Functional characterisation of PREL-1 and PREL-3 proteins as putative intramitochondrial phospholipid transporters governing homeostasis and organismal health inFrontiers in molecular biosciences · 2026Article
- The Core MICOS Complex Subunit mic60 has Been Substituted by Two Cryptic Mitofilin-containing Proteins in Euglenozoa.Molecular biology and evolution · 2025Article
- The CHCHD2-CHCHD10 protein complex is modulated by mitochondrial dysfunction and alters lipid homeostasis in the mouse brain.Cell death & disease · 2025Article
- CHCHD4 Oxidoreductase Activity: A Comprehensive Analysis of the Molecular, Functional, and Structural Properties of Its Redox-Regulated Substrates.Molecules (Basel, Switzerland) · 2025Review
- Origin and evolution of mitochondrial inner membrane composition.Journal of cell science · 2025Review
- The role of mitochondria-associated ER membranes in disease pathology: protein complex and therapeutic targets.Frontiers in cell and developmental biology · 2025Review
- SLMO transfers phosphatidylserine between the outer and inner mitochondrial membrane in Drosophila.PLoS biology · 2024Article
- Role of Yme1 in mitochondrial protein homeostasis: from regulation of protein import, OXPHOS function to lipid synthesis and mitochondrial dynamics.Biochemical Society transactions · 2024Review
- The phospholipids cardiolipin and phosphatidylethanolamine differentially regulate MDC biogenesis.The Journal of cell biology · 2024Article
- SLMO2 is a potential prognostic and immunological biomarker in human pan-cancer.Scientific reports · 2024Article
- Mic19 depletion impairs endoplasmic reticulum-mitochondrial contacts and mitochondrial lipid metabolism and triggers liver disease.Nature communications · 2024Article
- Mitochondrial phospholipid metabolism in health and disease.Journal of cell science · 2023Article
- Quantifying organellar ultrastructure in cryo-electron tomography using a surface morphometrics pipeline.The Journal of cell biology · 2023Article
- Article
- A CHCHD6-APP axis connects amyloid and mitochondrial pathology in Alzheimer's disease.Acta neuropathologica · 2022Article
- Protocol for engineering and validating a synthetic mitochondrial intermembrane bridge in mammalian cells.STAR protocols · 2022Article
25 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
8 authors at 4 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Mitochondria exert critical functions in cellular lipid metabolism and promote the synthesis of major constituents of cellular membranes, such as phosphatidylethanolamine (PE) and phosphatidylcholine. Here, we demonstrate that the phosphatidylserine decarboxylase Psd1, located in the inner mitochondrial membrane, promotes mitochondrial PE synthesis via two pathways. First, Ups2-Mdm35 complexes (SLMO2-TRIAP1 in humans) serve as phosphatidylserine (PS)-specific lipid transfer proteins in the mitochondrial intermembrane space, allowing formation of PE by Psd1 in the inner membrane. Second, Psd1 decarboxylates PS in the outer membrane in trans, independently of PS transfer by Ups2-Mdm35. This latter pathway requires close apposition between both mitochondrial membranes and the mitochondrial contact site and cristae organizing system (MICOS). In MICOS-deficient cells, limiting PS transfer by Ups2-Mdm35 and reducing mitochondrial PE accumulation preserves mitochondrial respiration and cristae formation. These results link mitochondrial PE metabolism to MICOS, combining functions in protein and lipid homeostasis to preserve mitochondrial structure and function.
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