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Local accumulation of very long-chain PUFA in plexiform layers associates with retinal dysfunction in a mouse model of peroxisomal ACBD5-deficiency.

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Title: Local accumulation of very long-chain PUFA in plexiform layers associates with retinal dysfunction in a mouse model of peroxisomal ACBD5-deficiency.
Authors: Merz J; Institute of Neuroanatomy, Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany.; Mannheim Center for Translational Neuroscience (MCTN), Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany., Müller E; Center for Mass Spectrometry and Optical Spectroscopy (CeMOS), Technische Hochschule Mannheim, Mannheim, Germany.; Medical Faculty Heidelberg, Heidelberg University, Heidelberg, Germany., Darwisch W; Institute of Neuroanatomy, Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany.; Mannheim Center for Translational Neuroscience (MCTN), Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany., Fairless R; Department of Neurology, University Clinic Heidelberg, Heidelberg, Germany.; Clinical Cooperation Unit (CCU) Neurooncology German Cancer Consortium (DKTK), German Cancer Research Center (DFKZ), Heidelberg, Germany., Wang Y; Experimental Pharmacology, European Center for Angioscience (ECAS), Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany., Vorwald S; Institute of Neuroanatomy, Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany.; Mannheim Center for Translational Neuroscience (MCTN), Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany., Darwisch S; Institute of Neuroanatomy, Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany.; Mannheim Center for Translational Neuroscience (MCTN), Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany., Curticean ER; BioQuant, Medical Faculty Heidelberg, Heidelberg University, Heidelberg, Germany., Shao F; Experimental Pharmacology, European Center for Angioscience (ECAS), Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany., Wacker I; BioQuant, Medical Faculty Heidelberg, Heidelberg University, Heidelberg, Germany., Schröder RR; BioQuant, Medical Faculty Heidelberg, Heidelberg University, Heidelberg, Germany., Pitzer C; Interdisciplinary Neurobehavioral Core, INBC, Ruprecht-Karls-Universität Heidelberg, Heidelberg, Germany., Schultz C; Institute of Neuroanatomy, Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany.; Mannheim Center for Translational Neuroscience (MCTN), Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany., van Klinken JB; Department of Laboratory Medicine and Pediatrics, Laboratory Genetic Metabolic Diseases, Amsterdam UMC location University of Amsterdam, Emma Children's Hospital, Amsterdam, The Netherlands.; Core Facility Metabolomics, Amsterdam UMC Location University of Amsterdam, Amsterdam, The Netherlands.; Department of Human Genetics, Leiden University Medical Center, Leiden, Netherlands., Vaz FM; Department of Laboratory Medicine and Pediatrics, Laboratory Genetic Metabolic Diseases, Amsterdam UMC location University of Amsterdam, Emma Children's Hospital, Amsterdam, The Netherlands.; Department of Human Genetics, Leiden University Medical Center, Leiden, Netherlands.; Amsterdam Gastroenterology Endocrinology Metabolism, Inborn errors of metabolism, Amsterdam, The Netherlands., Kratzer F; Department of General Pediatrics, Division of Neuropediatrics and Metabolic Medicine, University Hospital Heidelberg Center of Pediatric and Adolescent Medicine, Heidelberg, Germany., Schwarz K; Department of General Pediatrics, Division of Neuropediatrics and Metabolic Medicine, University Hospital Heidelberg Center of Pediatric and Adolescent Medicine, Heidelberg, Germany., Okun JG; Department of General Pediatrics, Division of Neuropediatrics and Metabolic Medicine, University Hospital Heidelberg Center of Pediatric and Adolescent Medicine, Heidelberg, Germany., Feng Y; Experimental Pharmacology, European Center for Angioscience (ECAS), Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany., Hopf C; Mannheim Center for Translational Neuroscience (MCTN), Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany.; Center for Mass Spectrometry and Optical Spectroscopy (CeMOS), Technische Hochschule Mannheim, Mannheim, Germany.; Medical Faculty Heidelberg, Heidelberg University, Heidelberg, Germany., Islinger M; Institute of Neuroanatomy, Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany. markus.islinger@medma.uni-heidelberg.de.; Mannheim Center for Translational Neuroscience (MCTN), Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany. markus.islinger@medma.uni-heidelberg.de.; Institute of Neuroanatomy, Mannheim Center for Translational Neuroscience (MCTN), Medical Faculty Mannheim, Heidelberg University, Ludolf-Krehl Str. 13-17, Mannheim, 68167, Germany. markus.islinger@medma.uni-heidelberg.de.
Source: Cellular and molecular life sciences : CMLS [Cell Mol Life Sci] 2025 Dec 01; Vol. 83 (1), pp. 26. Date of Electronic Publication: 2025 Dec 01.
Publication Type: Journal Article
Journal Info: Publisher: Springer Country of Publication: Switzerland NLM ID: 9705402 Publication Model: Electronic Cited Medium: Internet ISSN: 1420-9071 (Electronic) Linking ISSN: 1420682X NLM ISO Abbreviation: Cell Mol Life Sci Subsets: MEDLINE
Database: MEDLINE Ultimate
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ISSN:1420-9071
DOI:10.1007/s00018-025-05971-8