Cited by 65Metabolism and Genetic DisordersHereditary Neurological DisordersLysosomal Storage Disorders Research

Peroxisomes, myelination, and axonal integrity in the CNS

Baes, Myriam, Aubourg, Patrick·2009·The Neuroscientist

Abstract

Peroxisomes are ubiquitous organelles with multiple metabolic functions, but their precise role in the maintenance of tissues is not well understood. All diseases caused by partial or complete peroxisome dysfunction are characterized by a variety of neurological abnormalities, underscoring the importance of peroxisomes in nervous tissue. The interrelationship between metabolic abnormalities, histological changes, and clinical signs in these peroxisomal diseases has not yet been clarified. During the past decade, a more systematic study of the consequences of peroxisome dysfunction was possible through the generation of knockout mice with generalized or conditional inactivation of peroxisomal proteins. It appears that peroxisomes are necessary for the preservation of axonal integrity and for the formation and maintenance of myelin.

Integrity signals

Registry

Crossref · 10.1177/1073858409336297

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132 of 132 references verified0 citation errors · 0 not checkable

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  • In some PBD cases, mild initial symptoms are later followed by severe CNS demyelination and death.

    “In some cases, mild initial symptoms are later followed by severe CNS demyelination and death of the patient [53,55,56].”

    From Peroxisomes in brain development and function (2016) · cites this paper as [56]

  • Because myelination is still ongoing during the early period, it cannot be clearly established whether the lack of peroxisomal functions causes abnormal myelination, early demyelination or both processes simultaneously.

    “Because myelination is still ongoing during this early period, it cannot be clearly established, whether the lack of peroxisomal functions causes abnormal myelination, early demyelination or both processes simultaneously [56].”

    From Peroxisomes in brain development and function (2016) · cites this paper as [56]

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Authors
Baes, M., & Aubourg, P.
Affiliations
  • KU Leuven (BE)
  • Inserm (FR)
Corresponding author
Baes, Myriam
Venue
The Neuroscientist
Published
2009-08
Volume
15
Issue
4
Pages
367-379
ISSN
  • 1073-8584 (ISSN-L)
  • 1089-4098
OpenAlex
W2152692214
Cited by
65
References
132
Citations by year
2026: 2 · 2025: 3 · 2022: 1 · 2021: 1 · 2020: 3 · 2019: 2 · 2018: 2 · 2017: 5 · 2016: 3 · 2015: 6 · 2014: 4 · 2013: 8 · 2012: 13
FWCI
0.99
Type
journal article
Language
en
Open access
closed
Locations
  • The Neuroscientist (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
  • Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
Keywords
peroxisomes, peroxisomal disorders, myelination, axonal integrity, central nervous system, knockout mice, peroxisomal proteins, myelin maintenance, axon maintenance
MeSH terms
Animals, Axons, Central Nervous System, Demyelinating Diseases, Humans, Lipid Metabolism, Inborn Errors, Myelin Proteins, Myelin Sheath, Peroxisomes, Lipid Metabolism

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