title: Myelin peroxisomes - essential organelles for the maintenance of white matter in the nervous system.
abstract: Peroxisomes are cellular compartments primarily associated with lipid metabolism. Most cell types, including nervous system cells, harbor several hundred of these organelles. The importance of peroxisomes for central nervous system white matter is evidenced by a variety of human peroxisomal disorders with neurological impairment frequently involving the white matter. Moreover, the most frequent childhood white matter disease, X-linked adrenoleukodystrophy, is a peroxisomal disorder. During the past decade advances in imaging techniques have enabled the identification of peroxisomes within the myelin sheath, especially close to nodes of Ranvier. Although the function of myelin peroxisomes is not solved yet on molecular level, recently acquired knowledge suggests a central role for these organelles in axo-glial metabolism. This review focuses on the biology of myelin peroxisomes as well as on the pathology of myelin and myelinated axons that is observed as a consequence of partial or complete peroxisomal dysfunction in the brain.
authors.affiliation: Department of Neurogenetics, Max Planck Institute of Experimental Medicine, Hermann-Rein-Straße 3, 37075 Göttingen, Germany. Electronic address: Kassmann@em.mpg.de.
authors.forename: Celia M
authors.initials: CM
authors.lastname: Kassmann
mesh_terms: D000326:Adrenoleukodystrophy / Q000473:pathology / Q000503:physiopathology; D000818:Animals; D001369:Axons / Q000378:metabolism; D006801:Humans; D051379:Mice; D009186:Myelin Sheath / Q000378:metabolism*; D009457:Neuroglia; D018901:Peroxisomal Disorders / Q000473:pathology; D020675:Peroxisomes / Q000378:metabolism*
keywords: Adrenoleukodystrophy; Axoglial metabolism; Myelin; Peroxisomes