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Axons need glial peroxisomes

Nature Genetics volume 39, pages 936–938 (2007)Cite this article

Several devastating genetic diseases illustrate that peroxisomes are essential to the development and functioning of the central nervous system. New work using a mouse model now shows that peroxisome integrity in oligodendrocytes is essential for axonal maintenance.

First described by Baudhuin and de Duve in the mid-1950s, peroxisomes are single, membrane-bound, typically spherical organelles ranging in size from 0.1 μm to 1 μm in diameter and ranging in number from a few hundred to a few thousand per mammalian cell. The dense, amorphous peroxisome matrix contains more than 50 enzymes that participate in the β-oxidation of 2-methyl branched-chain and very-long-chain fatty acids, the synthesis of ether lipids (plasmalogens) and the oxidation of D-amino acids and polyamines. H2O2 produced by peroxisomal oxidases is detoxified in situ by peroxisomal catalase. The machinery that imports peroxisomal enzymes into the peroxisomal matrix is highly flexible and includes the cycling receptor peroxin 5, encoded by Pex5. Genetic defects of PEX genes in humans result in severe brain malformation and hypomyelination (Zellweger syndrome)1.

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Figure 1: Oligodendrocyte peroxisomes.

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  1. Department of Pediatric Neurology, Patrick Aubourg is at INSERM U745, Hôpital Saint-Vincent de Paul, University René Descartes, Paris 75014, France. aubourg@paris5.inserm.fr,

    Patrick Aubourg

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Aubourg, P. Axons need glial peroxisomes. Nat Genet 39, 936–938 (2007). https://doi.org/10.1038/ng0807-936

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