Fetal adrenoleukodystrophy: the significance of pathologic lesions in adrenal gland and testis
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Cholesterol along with saturated VLCFAs accumulates in the zona fasciculata and reticularis of the adrenal glands.
“However, it has been observed that cholesterol, along with saturated VLCFAs, accumulates in the zona fasciculata and reticularis of the adrenal glands.”
From X-linked adrenoleukodystrophy and primary adrenal insufficiency (2023) · cites this paper as [18]
This accumulation starts during fetal development.
“This accumulation starts during fetal development (18).”
From X-linked adrenoleukodystrophy and primary adrenal insufficiency (2023) · cites this paper as [18]
The accumulation of cholesterol and saturated VLCFAs in the zona fasciculata and reticularis of the adrenal cortex starts during foetal life.
“It has been demonstrated that the accumulation of cholesterol and saturated VLCFAs in the zona fasciculata and reticularis of the adrenal cortex starts during foetal life (18), probably in conjunction with the activation of foetal adrenal steroidogenesis.”
From X-linked adrenoleukodystrophy and primary adrenal insufficiency (2023) · cites this paper as [18]
These accumulations start during fetal development.
“These accumulations start during fetal development [424].”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [424]
Cites
Adrenoleukodystrophy: Electron Microscopic Findings
Powell, H. (1975) · Archives of Neurology · doi:10.1001/archneur.1975.00490460066009 · Cited by 76 · 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
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A mouse model for X-linked adrenoleukodystrophy
Lu, J.-F., Lawler, A. M., Watkins, P. A., Powers, J. M., Moser, A. B., Moser, H. W., & Smith, K. D. (1997) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.94.17.9366 · Cited by 293 · see citing works
Self-citationAdrenoleukodystrophy—Neuroendocrine pathogenesis and redefinition of natural history
Kemp, S., Huffnagel, I. C., Linthorst, G. E., Wanders, R. J., & Engelen, M. (2016) · Nature Reviews Endocrinology · doi:10.1038/nrendo.2016.90 · Cited by 275 · see citing works
Pathophysiology of X-linked adrenoleukodystrophy
Berger, J., Forss-Petter, S., & Eichler, F. S. (2014) · Biochimie · doi:10.1016/j.biochi.2013.11.023 · Cited by 238 · see citing works
Peroxisomal Disorders: Genotype, Phenotype, Major Neuropathologic Lesions, and Pathogenesis
Powers, J. M., & Moser, H. W. (1998) · Brain Pathology · doi:10.1111/j.1750-3639.1998.tb00139.x · Cited by 208 · see citing works
Self-citationDubey, P., Raymond, G. V., Moser, A. B., Kharkar, S., Bezman, L., & Moser, H. W. (2005) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2004.10.067 · Cited by 145 · see citing works
Self-citationAubourg, P., Scotto, J., Rocchiccioli, F., Feldmann-Pautrat, D., & Robain, O. (1986) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp.49.1.77 · Cited by 77 · see citing works
X-linked adrenoleukodystrophy and primary adrenal insufficiency
Cappa, M., Todisco, T., & Bizzarri, C. (2023) · Frontiers in Endocrinology · doi:10.3389/fendo.2023.1309053 · Cited by 28 · 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
- Powers, J. M., Moser, H. W., Moser, A. B., & Schaumburg, H. H.
- Corresponding author
- Powers, James M.
- Venue
- Human Pathology
- Publisher
- Elsevier BV
- Published
- 1982-11
- Volume
- 13
- Issue
- 11
- Pages
- 1013-1019
- ISSN
- 0046-8177 (ISSN-L)
- OpenAlex
- W1967473148
- Cited by
- 49
- References
- 2
- Citations by year
- 2026: 1 · 2025: 2 · 2023: 2 · 2022: 3 · 2021: 2 · 2020: 1 · 2019: 2 · 2016: 3 · 2014: 1 · 2013: 1 · 2012: 1
- FWCI
- 0.44
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Human Pathology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- School of Medicine, University of New Mexico
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and rare skin diseases.
- Keywords
- adrenoleukodystrophy, adrenal gland, testis, Leydig cells, adrenocortical cells, lamellar inclusions, striations, amniotic fluid cells, abortus, family history (medicine), electron microscopy
- MeSH terms
- Abortion, Therapeutic, Adrenal Cortex, Adrenal Glands, Adrenoleukodystrophy, Diffuse Cerebral Sclerosis of Schilder, Fatty Acids, Female, Fetal Diseases, Humans, Leydig Cells, Male, Pregnancy, Testis
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