Peroxisomal and mitochondrial status of two murine oligodendrocytic cell lines (158N, 158JP): potential models for the study of peroxisomal disorders associated with dysmyelination processes.

6533b7defe1ef96bd1275e74

RESEARCH PRODUCT

Peroxisomal and mitochondrial status of two murine oligodendrocytic cell lines (158N, 158JP): potential models for the study of peroxisomal disorders associated with dysmyelination processes.

Franck Ménétrier Mauhamad Baarine Pierre Andreoletti Emmanuelle C. Genin Moustapha Cherkaoui-malki Doriane Trompier M. Said Ghandour Gérard Lizard Kévin Ragot Stéphane Savary Hammam I. El Hajj

subject

Proteolipid protein 1 Biochemistry Mice Myelin MESH : Phenylbutyrates peroxisome Isomerases MESH : Myelin Basic Proteins Enoyl-CoA Hydratase Cell Line Transformed Ultrasonography biology MESH : Gene Expression Regulation MESH : Myelin Proteolipid Protein 3-Hydroxyacyl CoA Dehydrogenases MESH : Myelin-Associated Glycoprotein MESH : Cell Line Transformed Peroxisome MESH : Multienzyme Complexes MESH : Oligodendroglia MESH : Enoyl-CoA Hydratase Catalase Flow Cytometry MESH : 3-Hydroxyacyl CoA Dehydrogenases Phenylbutyrates mitochondria Myelin-Associated Glycoprotein Oligodendroglia myelin MESH : Antineoplastic Agents medicine.anatomical_structure MESH : Microscopy Electron Transmission Biochemistry ACOX1 MESH : Mitochondria MESH : Acyl-CoA Oxidase 2'3'-Cyclic-Nucleotide Phosphodiesterases MESH : Isomerases Oxidation-Reduction Myelin Proteins MESH : Flow Cytometry Antineoplastic Agents Peroxisomal Bifunctional Enzyme Statistics Nonparametric Myelin oligodendrocyte glycoprotein Cellular and Molecular Neuroscience Microscopy Electron Transmission Multienzyme Complexes MESH : Catalase MESH : Mice Peroxisomes medicine Animals MESH : ATP-Binding Cassette Transporters Myelin Proteolipid Protein MESH : Statistics Nonparametric [ SDV.BBM ] Life Sciences [q-bio]/Biochemistry Molecular Biology MESH : Oxidation-Reduction Myelin Basic Protein murine oligodendrocytes MESH : 2'3'-Cyclic-Nucleotide Phosphodiesterases Peroxisomal transport Oligodendrocyte Myelin basic protein Gene Expression Regulation biology.protein ATP-Binding Cassette Transporters Myelin-Oligodendrocyte Glycoprotein Acyl-CoA Oxidase MESH : Animals MESH : Peroxisomes

description

International audience; In some neurodegenerative disorders (leukodystrophies) characterized by myelin alterations, the defect of peroxisomal functions on myelin-producing cells (oligodendrocytes) are poorly understood. The development of in vitro models is fundamental to understanding the physiopathogenesis of these diseases. We characterized two immortalized murine oligodendrocyte cell lines: a normal (158N) and a jimpy (158JP) cell line mutated for the proteolipid protein PLP/DM20. Fluorescence microscopy, flow cytometry, and western blotting analysis allow to identify major myelin proteins (PLP colocalizing with mitochondria; myelin basic protein), oligodendrocyte (CNPase and myelin oligodendrocyte glycoprotein), and peroxisomal markers [adrenoleukodystrophy protein, PMP70, acyl-CoA oxidase 1 (ACOX1), l-peroxisomal bifunctional enzyme, and catalase]. Using electron microscopy, peroxisomes were identified in the two cell lines. Gene expression (ATP-binding cassette, Abcd1, Abcd2, Abcd3, and Acox1) involved in peroxisomal transport or beta-oxidation of fatty acids was evaluated using quantitative PCR. 4-phenylbutyrate treatment increases expression of ACOX1, l-peroxisomal bifunctional enzyme, PLP, myelin oligodendrocyte glycoprotein, and CNPase, mainly in 158N cells. In both cell lines, 4-phenylbutyrate-induced ACOX1 and catalase activities while only Abcd2 gene was up-regulated in 158JP. Moreover, the higher mitochondrial activity and content observed in 158JP were associated with higher glutathione content and increased basal production of reactive oxygen species revealing different redox statuses. Altogether, 158N and 158JP cells will permit studying the relationships between peroxisomal defects, mitochondrial activity, and oligodendrocyte functions.

year	journal	country	edition	language
2009-10-01

https://hal.archives-ouvertes.fr/hal-00514890/document