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Cited by 137Metabolism and Genetic DisordersLipid metabolism and biosynthesisLysosomal Storage Disorders Research

The DOI matches its Crossref record; 33 of the 33 references it cites were found in a registry or an index.

FATTY ACID ABNORMALITY IN ADRENOLEUKODYSTROPHY

Igarashi, M., Schaumburg, H. H., Powers, J., Kishimoto, Y., Koilodny, E., Suzuki, K.·1976·Journal of Neurochemistry
DOI 10.1111/j.1471-4159.1976.tb04461.x-i1
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Abstract

—Recent clinical and morphological evidence established that adrenoleukodystrophy is a distinct X‐linked genetic disorder. Fatty acid compositions of lipids in the brain, adrenal and serum from seven patients were examined. Cholesterol esters of both brain and adrenal contained substantial proportions of fatty acids longer than C 22 (11.8–41.9% of total in the brain and 13.4‐34.8% of total in the adrenal), while cholesterol esters from normal and pathological control specimens contained very little. These very long chain fatty acids were generally saturated in brain cholesterol esters but significant amounts of unsaturated long chain fatty acids were also present in adrenal cholesterol esters. The long chain fatty acids showed bell‐shaped distribution with C 25 or C 26 at the peak. Ganglio‐sides from patients’white matter also showed increased proportions of very long‐chain fatty acids, up to 50% of the total. Qualitatively similar but much milder fatty acid abnormalities were also found in galactosylceramide of the brain. On the other hand, fatty acids and fatty aldehydes of brain glycerophospholipids, adrenal free fatty acids, triglycerides and glycerophospholipids were not abnormal. Furthermore, serum cholesterol esters from two patients did not show the long‐chain fatty acid abnormality found in brain and adrenal cholesterol esters. Sequential extractions with acetone and hexane established that the characteristic birefringent material in the brain and adrenal is indeed cholesterol esters with very long chain fatty acids. This type of fatty acid abnormality has not been described in other pathological conditions and may well represent the unique biochemical abnormality that is directly related to the fundamental genetic defect underlying adrenoleukodystrophy.

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Crossref · 10.1111/j.1471-4159.1976.tb04461.x-i1

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Cites

Research works printed but not linked: 4

  1. Glomset J. A., 1964, Biochim. biophys. Acta, 89, 266
  2. Norman R. M., 1963, Brain Lipids and Liproproteins, and the Leucodystrophies, 169
  3. Powers J. M., 1973, Archs. Path., 96, 305
  4. Powers J. M., 1974, Am. J. Path., 76, 481

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Metadata

Authors
Igarashi, M., Schaumburg, H. H., Powers, J., Kishimoto, Y., Koilodny, E., & Suzuki, K.
Affiliations
Albert Einstein College of Medicine (US)
Corresponding author
Suzuki, K.
ORCID
Powers, J.
Venue
Journal of Neurochemistry
Publisher
Wiley
Published
1976-04
Volume
26
Issue
4
Pages
851-860
ISSN
  • 0022-3042 (ISSN-L)
  • 1471-4159
DOI
10.1111/j.1471-4159.1976.tb04461.x-i1
OpenAlex
W4232666095
Cited by
134
References
33
Citations by year
2026: 3 · 2025: 6 · 2024: 12 · 2023: 1 · 2022: 5 · 2021: 7 · 2020: 5 · 2019: 1 · 2018: 2 · 2017: 3 · 2016: 4 · 2014: 2 · 2013: 1 · 2012: 1
FWCI
2.73
Type
journal article
Language
en
Open access
closed
Licence
onlinelibrary.wiley.com/termsAndConditions#vor (vor, 11143 d)
Locations
Journal of Neurochemistry (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Lipid metabolism and biosynthesis
  • Health Sciences › Medicine › Genetics (clinical) › Lysosomal Storage Disorders Research
Keywords
adrenoleukodystrophy, very long chain fatty acids, cholesterol esters, fatty acid composition, brain lipids, gangliosides, galactosylceramide, X-linked disorders, white matter pathology, birefringent materials

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