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Cited by 23Multiple Sclerosis Research StudiesLysosomal Storage Disorders ResearchMetabolism and Genetic Disorders

Concurrent axon and myelin destruction differentiates X-linked adrenoleukodystrophy from multiple sclerosis

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Bergner, Caroline G., Genc, Nafiye, Hametner, Simon, Franz, Jonas, van der Meer, Franziska, Mitkovski, Miso, Weber, Martin S., Stoltenburg‐Didinger, Gisela +5 more·2021·Glia
DOI 10.1002/glia.24042
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Abstract

Cerebral disease manifestation occurs in about two thirds of males with X‐linked adrenoleukodystrophy (CALD) and is fatally progressive if left untreated. Early histopathologic studies categorized CALD as an inflammatory demyelinating disease, which led to repeated comparisons to multiple sclerosis (MS). The aim of this study was to revisit the relationship between axonal damage and myelin loss in CALD. We applied novel immunohistochemical tools to investigate axonal damage, myelin loss and myelin repair in autopsy brain tissue of eight CALD and 25 MS patients. We found extensive and severe acute axonal damage in CALD already in prelesional areas defined by microglia loss and relative myelin preservation. In contrast to MS, we did not observe selective phagocytosis of myelin, but a concomitant decay of the entire axon‐myelin unit in all CALD lesion stages. Using a novel marker protein for actively remyelinating oligodendrocytes, breast carcinoma‐amplified sequence (BCAS) 1, we show that repair pathways are activated in oligodendrocytes in CALD. Regenerating cells, however, were affected by the ongoing disease process. We provide evidence that—in contrast to MS—selective myelin phagocytosis is not characteristic of CALD. On the contrary, our data indicate that acute axonal injury and permanent axonal loss are thus far underestimated features of the disease that must come into focus in our search for biomarkers and novel therapeutic approaches.

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  • BCAS1+ oligodendrocytes in the cortex appear in two main phenotypes: resting cells with few or no immunopositive processes, prevailing in the healthy adult brain, and activated cells with pre-myelinating/myelinating morphology characterized by internode formation.

    “BCAS1+ oligodendrocytes in the cortex appear in two main phenotypes: cells with few or no immunopositive processes, prevailing in the healthy adult brain (‘resting’ BCAS1+ oligodendrocytes; arrows in Fig. 1A) and cells with a pre-myelinating morphology with multiple branches and myelinating morphology, characterized by internode formation (‘activated’ BCAS1+ oligodendrocytes; arrowheads in Fig. 1A”

    From BCAS1-positive oligodendrocytes enable efficient cortical remyelination in multiple sclerosis (2025) · cites this paper as [37]

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  • In many neurodegenerative diseases, including AMN, axonal pathology precedes cell body loss in the form of dying back axonopathy.

    “In many neurodegenerative diseases, including AMN [49,89,181,187,288,294,295,296,297], axonal pathology precedes cell body loss in the form of “dying back axonopathy”, in which axons gradually degenerate toward the cell body [298,299].”

    From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [297]

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Cited by

Sorted by citations

Authors
Bergner, C. G., Genc, N., Hametner, S., Franz, J., van der Meer, F., Mitkovski, M., Weber, M. S., Stoltenburg‐Didinger, G., Kühl, J., Köhler, W., Brück, W., Gärtner, J., & Stadelmann, C.
Affiliations
  • Universitätsmedizin Göttingen (DE)
  • Medical University of Vienna (AT)
  • Institute of Neuropathology, University Medical Center Göttingen Göttingen Germany
  • Light Microscopy Facility Max‐Planck Institute for Experimental Medicine Göttingen Germany
  • Institute of Cell Biology and Neurobiology, Charité University Medicine Berlin Germany
  • University Hospital Leipzig (DE)
Corresponding author
Stadelmann, Christine
ORCID
Venue
Glia
Publisher
Wiley
Published
2021-06-17
Volume
69
Issue
10
Pages
2362-2377
ISSN
  • 0894-1491 (ISSN-L)
  • 1098-1136
DOI
10.1002/glia.24042
OpenAlex
W3168152314
Cited by
23
References
17
Citations by year
2026: 3 · 2025: 4 · 2024: 8 · 2023: 5 · 2022: 1 · 2021: 2
FWCI
2.67
Type
journal article
Language
en
Open access
Licence
Locations
  • Glia (publishedVersion, cc-by-nc-nd)
  • PubMed (publishedVersion)
  • GoeScholar The Publication Server of the Georg-August-Universität Göttingen (Georg-August-Universität Göttingen) (submittedVersion, other-oa)
Funders
  • Association Européenne contre les Leucodystrophies
  • Deutsche Forschungsgemeinschaft
  • Deutschen Multiple Sklerose Gesellschaft
  • Gemeinnützige Hertie-Stiftung
  • National Multiple Sclerosis Society
Topics
  • Health Sciences › Medicine › Pathology and Forensic Medicine › Multiple Sclerosis Research Studies
  • Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
Keywords
X-linked adrenoleukodystrophy, multiple sclerosis, axonal injury, demyelination, myelin phagocytosis, remyelination, oligodendrocytes, BCAS1, demyelinating disease, biomarkers
MeSH terms
Adrenoleukodystrophy, Axons, Humans, Male, Multiple Sclerosis, Myelin Sheath, Oligodendroglia

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