Peripheral Neuropathy in the Adreno-myelo-neuropathy Mouse Model
Open accessAbstract
The Abcd1 knockout mouse mimics human adreno-myelo-neuropathy (AMN), thus contributes to a better understanding of disease mechanisms, which yet remain poorly identified. Only limited information is available about peripheral neuropathy (PN), although a notable component of AMN pathology besides myelopathy. To enrich our knowledge of PN, the current study reports the clinical, electromyographic and morphological aspects of peripheral neuropathy. We found that despite obvious electron microscopy anomalies in sciatic nerve axons, nerve conduction was nearly normal and did not seem to contribute significantly to the impaired motor performances of the Abcd1-/- mouse.
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Abcd1-knockout mice do not develop peripheral neuropathy.
“VLCFA accumulation occurs, but not brain lesions [26,27,28,29] or peripheral neuropathy [175].”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [175]
Unlike other authors, we did not find nerve conduction defects in our Abcd1-/- mice.
“However, unlike other authors [29], we did not find nerve conduction defects in our Abcd1−/− mice [175].”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [175]
Cites
Pujol, A. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.5.499 · Cited by 214 · see citing works
Adenoassociated virus serotype 9-mediated gene therapy for x-linked adrenoleukodystrophy
Gong, Y., Mu, D., Prabhakar, S., Moser, A., Musolino, P., Ren, J., Breakefield, X. O., Maguire, C. A., & Eichler, F. S. (2015) · Molecular Therapy · doi:10.1038/mt.2015.6 · Cited by 74 · see citing works
Adrenomyeloneuropathy: A protracted, pseudosystematic variant of adrenoleukodystrophy
Probst, A., Ulrich, J., Heitz, P. U., & Herschkowitz, N. (1980) · Acta Neuropathologica · doi:10.1007/bf00690750 · Cited by 36 · see citing works
Adrenomyeloneuropathy: A report on two families
Martin, J. J., Lowenthal, A., Ceuterick, C., & Gacoms, H. (1982) · Journal of Neurology · doi:10.1007/bf00313395 · Cited by 27 · see citing works
Özgür-Günes, Y., Chedik, M., Le Stunff, C., Fovet, C.-M., & Bougnères, P. (2022) · Human Gene Therapy · doi:10.1089/hum.2021.293 · Cited by 10 · see citing works
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Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy
Bougnères, P., & Le Stunff, C. (2025) · Genes · doi:10.3390/genes16050590 · Cited by 11 · see citing works
- Authors
- Özgür Günes, Y., Le Stunff, C., Vallat, J., & Bougnères, P.
- Affiliations
- Centre Hospitalier Universitaire de Limoges (FR)
- Venue
- bioRxiv (Cold Spring Harbor Laboratory)
- Publisher
- openRxiv
- Published
- 2024-06-01
- OpenAlex
- W4399253607
- Cited by
- 2
- References
- 5
- Citations by year
- 2025: 2
- Type
- posted content
- Language
- en
- Open access
- green
- doi.org
- Licence
- cc-by-nc-nd
- Locations
- bioRxiv (Cold Spring Harbor Laboratory) (acceptedVersion, cc-by-nc-nd)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
- Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
- Health Sciences › Medicine › Neurology › Peripheral Neuropathies and Disorders
- Keywords
- peripheral neuropathy, nerve conduction, electromyography, motor performance, myelopathy
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