The correlation between biochemical and histopathological findings in adrenoleukodystrophy
Abstract
Nine areas of the brain from a case of adrenoleukodystrophy were examined histopathologically and by gas chromatography for fatty acid content. The main findings were: (1) the degree of demyelination was related to the pentacosanoic and hexacosanoic to docosanoic acid ratios (C25:0/C22:0 and C26:0/C22:0); gliosis was related to the ratios of several fatty acids to docosanoic acid; (2) there was a shift towards smaller components (C22:0, C23:0, C24:0) of the saturated fatty acid series in the less affected areas, to larger components and various minor components in regions of active demyelination; (3) mainly saturated fatty acids of the middle class components (C24:0, C25:0 and C26:0) were found in severely affected areas where the active process is complete. Because a region of high long chain fatty acid content, lacking histopathological change, was detected, the hypothesis is presented that the primary event in childhood ALD is related to defective lipid metabolism and that this preceeds demyelination.
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cALD brain histopathology shows marked regional disparity with contiguous white matter areas ranging from intact myelinated axons to chronic gliotic lesions.
“Histopathology of five brains showed marked disparity across regions [72] with contiguous white matter areas [73]: (i) intact zones showing densely packed myelinated axons and normal-looking oligodendrocytes (OLs); (ii) zones of limited demyelination showing occasional astrocytes and lipid-containing macrophages; (iii) zones of active demyelination with loss of axons and myelin, variable loss of O”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [73]
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Kishimoto, Y., Moser, H. W., Kawamura, N., Platt, M., Pallante, S. L., & Fenselau, C. (1980) · Biochemical and Biophysical Research Communications · doi:10.1016/0006-291x(80)91182-1 · Cited by 79 · see citing works
TSUJI, S., SANO, T., ARIGA, T., & MIYATAKE, T. (1981) · The Journal of Biochemistry · doi:10.1093/oxfordjournals.jbchem.a133578 · Cited by 73 · see citing works
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Moser, H. W., Mahmood, A., & Raymond, G. V. (2007) · Nature Clinical Practice Neurology · doi:10.1038/ncpneuro0421 · Cited by 663 · see citing works
X-linked adrenoleukodystrophy: Clinical, metabolic, genetic and pathophysiological aspects
Kemp, S., Berger, J., & Aubourg, P. (2012) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2012.03.012 · Cited by 283 · see citing works
Very long chain fatty acids in higher animals: a review
Poulos, A. (1995) · Lipids · doi:10.1007/bf02537036 · Cited by 192 · see citing works
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
- Reinecke, C. J., Knoll, D. P., Pretorius, P. J., Steyn, H. S., & Simpson, R. H. W.
- Affiliations
- North-West University (ZA)
- University of the Witwatersrand (ZA)
- ORCID
- Pretorius, P.J.
- Venue
- Journal of the Neurological Sciences
- Publisher
- Elsevier BV
- Published
- 1985-08
- Volume
- 70
- Issue
- 1
- Pages
- 21-38
- ISSN
- 0022-510X (ISSN-L)
- OpenAlex
- W2053745906
- Cited by
- 29
- References
- 2
- Citations by year
- 2025: 1 · 2020: 1 · 2019: 1 · 2013: 1 · 2012: 3
- FWCI
- 1.33
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of the Neurological Sciences (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
- Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
- Keywords
- adrenoleukodystrophy, demyelination, brain fatty acid composition, long-chain fatty acids, docosanoic acid, hexacosanoic acid, gliosis, lipid metabolism, histopathology
- MeSH terms
- Adrenoleukodystrophy, Adult, Brain Chemistry, Brain Damage, Chronic, Brain Injuries, Diffuse Cerebral Sclerosis of Schilder, Child, Child, Preschool, Chromatography, Gas, Fatty Acids, Female, Gliosis, Humans, Infant, Inflammation, Leukodystrophy, Globoid Cell, Male, Multiple Sclerosis
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