Histochemical characteristics of the striated inclusions of adrenoleukodystrophy

A. B. Johnson, H. H. Schaumburg, J. M. Powers

Research output: Contribution to journalArticle

43 Citations (Scopus)

Abstract

The striated accumulations in adrenal cortical cells and brain macrophages that are characteristic of adrenoleukodystrophy were studied histochemically in cryostat sections to seek leads for the biochemical identification of the striated material. It stained pale pink with oil red O and did not stain with the Schultz cholesterol procedure or periodic acid Schiff technique. By utilizing the birefringence of the accumulations as a marker, it was determined that, unlike natural cholesterol and cholesterol esters, the striated material was resistant to acetone and ethanol extraction. It was readily soluble, however, in nonpolar solvents such as n hexane and chloroform. These findings indicated that the material was most probably a lipid, and suggested that sequential extraction of adrenoleukodystrophy adrenal and brain with acetone and then n hexane could be used to isolate this material in relatively pure form. Based on this lead, biochemical studies have just revealed a fatty acid abnormality in adrenoleukodystrophy which appears to be unique to this genetic disease.

Original languageEnglish (US)
Pages (from-to)725-730
Number of pages6
JournalJournal of Histochemistry and Cytochemistry
Volume24
Issue number6
StatePublished - 1976

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Adrenoleukodystrophy
Acetone
Cholesterol
Birefringence
Inborn Genetic Diseases
Periodic Acid
Cholesterol Esters
Brain
Chloroform
Ethanol
Coloring Agents
Fatty Acids
Macrophages
Lipids
n-hexane

ASJC Scopus subject areas

  • Cell Biology
  • Anatomy

Cite this

Histochemical characteristics of the striated inclusions of adrenoleukodystrophy. / Johnson, A. B.; Schaumburg, H. H.; Powers, J. M.

In: Journal of Histochemistry and Cytochemistry, Vol. 24, No. 6, 1976, p. 725-730.

Research output: Contribution to journalArticle

Johnson, A. B. ; Schaumburg, H. H. ; Powers, J. M. / Histochemical characteristics of the striated inclusions of adrenoleukodystrophy. In: Journal of Histochemistry and Cytochemistry. 1976 ; Vol. 24, No. 6. pp. 725-730.
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