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Restoration of lysosomal membrane integrity in cell models of Pompe disease depends on fatty acid synthase and its product palmitic acid.

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Title: Restoration of lysosomal membrane integrity in cell models of Pompe disease depends on fatty acid synthase and its product palmitic acid.
Authors: Le Guillou E; Institut Necker-Enfants Malades (INEM), Paris, 75015, France.; INSERM U1151/CNRS UMR 8253, Paris, 75015, France.; Université Paris Cité, Paris, 75006, France.; Biochemistry Laboratory, Necker-Enfants Malades Hospital, APHP, Paris, 75015, France., Segaloni A; Institut Necker-Enfants Malades (INEM), Paris, 75015, France.; INSERM U1151/CNRS UMR 8253, Paris, 75015, France.; Université Paris Cité, Paris, 75006, France., Gadault A; Institut Necker-Enfants Malades (INEM), Paris, 75015, France.; INSERM U1151/CNRS UMR 8253, Paris, 75015, France.; Université Paris Cité, Paris, 75006, France., Oliveira Dias C; Institut Necker-Enfants Malades (INEM), Paris, 75015, France.; INSERM U1151/CNRS UMR 8253, Paris, 75015, France.; Université Paris Cité, Paris, 75006, France., Henneman NF; Institut Necker-Enfants Malades (INEM), Paris, 75015, France.; INSERM U1151/CNRS UMR 8253, Paris, 75015, France.; Université Paris Cité, Paris, 75006, France., Su M; Institute of Pharmaceutical Sciences, Pharmaceutical (Bio-)Analysis, University of Tübingen, Auf Der Morgenstelle 8, Tübingen, 72076, Germany., Nemazanyy I; Platform for Metabolic Analyses, Structure Fédérative de Recherche Necker, INSERM US24/CNRS UAR 3633, Paris, 75015, France., Hifdi N; Institute of Cardiovascular and Metabolic Diseases (I2MC), INSERM-UMR 1297, University Paul Sabatier, Toulouse, France., Nivet-Antoine V; Biochemistry Laboratory, Necker-Enfants Malades Hospital, APHP, Paris, 75015, France., Laemmerhofer M; Institute of Pharmaceutical Sciences, Pharmaceutical (Bio-)Analysis, University of Tübingen, Auf Der Morgenstelle 8, Tübingen, 72076, Germany., Hnia K; Institute of Cardiovascular and Metabolic Diseases (I2MC), INSERM-UMR 1297, University Paul Sabatier, Toulouse, France., Caillaud C; Institut Necker-Enfants Malades (INEM), Paris, 75015, France.; INSERM U1151/CNRS UMR 8253, Paris, 75015, France.; Université Paris Cité, Paris, 75006, France.; Biochemistry Laboratory, Necker-Enfants Malades Hospital, APHP, Paris, 75015, France., Panasyuk G; Institut Necker-Enfants Malades (INEM), Paris, 75015, France. ganna.panasyuk@inserm.fr.; INSERM U1151/CNRS UMR 8253, Paris, 75015, France. ganna.panasyuk@inserm.fr.; Université Paris Cité, Paris, 75006, France. ganna.panasyuk@inserm.fr.
Source: Cellular & molecular biology letters [Cell Mol Biol Lett] 2026 Apr 09; Vol. 31 (1). Date of Electronic Publication: 2026 Apr 09.
Publication Type: Journal Article
Journal Info: Publisher: BioMed Central Country of Publication: England NLM ID: 9607427 Publication Model: Electronic Cited Medium: Internet ISSN: 1689-1392 (Electronic) Linking ISSN: 14258153 NLM ISO Abbreviation: Cell Mol Biol Lett Subsets: MEDLINE
Database: MEDLINE Ultimate
Description
ISSN:1689-1392
DOI:10.1186/s11658-026-00897-w