Peroxisomes, myelination, and axonal integrity in the CNS
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.
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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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Miao, K., Meng, M., Liu, L., Li, M., Bai, Y., Guo, Y., Zhang, G., Kong, J., & Mu, J. (2026) · Cellular and Molecular Neurobiology · doi:10.1007/s10571-026-01729-3 · Cited by 1 · see citing works
Baarine, M. (2010) · theses.fr (ABES) · doi:10.70675/7ab2bcc8z16d2z420cz92f9zb3fd52371382 · Cited by 1 · see citing works
Youssef, J., & Badr, M. Z. (2013) · Humana Press eBooks · doi:10.1007/978-1-62703-420-3_2
The role of Nrg1-ErbB signaling in promoting Schwann cell myelination
Syed, N. (2012) · Rutgers University Community Repository (Rutgers University) · doi:10.7282/t3n878ms
(2015) · Elsevier eBooks · doi:10.1016/b978-3-437-31618-0.16001-8
Peroxisome Biogenesis Disorders
Demers, N. D., & Kim, P. K. (2019) · Elsevier eBooks · doi:10.1016/b978-0-12-801238-3.65924-x
Olfactory dysfunction and submicroscopic abnormalities: unveiling hidden pathologies
Bilińska, K., Pasińska, M., & de Castro, F. (2026) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2026.1861373
- Authors
- Baes, M., & Aubourg, P.
- Affiliations
- KU Leuven (BE)
- Inserm (FR)
- Corresponding author
- Baes, Myriam
- ORCID
- Baes, Myriam
- Venue
- The Neuroscientist
- Publisher
- SAGE Publications
- 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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