A correlative study of the adrenal cortex in adreno-leukodystrophy

Evidence for a fatal intoxication with very long chain saturated fatty acids

J. M. Powers, H. H. Schaumburg, A. B. Johnson, C. S. Raine

Research output: Chapter in Book/Report/Conference proceedingChapter

71 Citations (Scopus)

Abstract

Thirty adrenal glands from patients with adreno-leukodystrophy (ALD) have been studied by light microscopy, three by enzyme histochemistry, three by electron microscopy and two by tissue culture. Cytoplasmic ballooning and striations result from proliferation of smooth endoplasmic reticulum and accumulations of lamellar-lipid profiles and clear clefts (crystalloids). Striated adrenocortical cells, the only pathognomonic adrenal lesion in ALD, display cytoplasmic lamellae, decreased amounts of rough endoplasmic reticulum and depression of several enzymes (alpha-glycerophosphate dehydrogenase, 3β-hydroxysteroid dehydrogenase and TPNH diaphorase). The striated cells also demonstrate decreased ability to adapt to changes in microenvironment, both in vivo and in vitro. A blunted response by striated cells to focal peripheral cytolysis leads to cytoplasmic erosion, atrophy and macrovacuoles. ACTH has a pivotal role in the evolution of these lesions. The authors propose that the pathognomonic lamellae of ALD basically represent bilayers or bimolecular leaflets of very long chain saturated fatty acids, while lamellar-lipid profiles and clefts contain cholesterol esterified to these abnormal fatty acids. The similarity of lamellar-lipid profiles of ALD to cytoplasmic lesions induced by long chain saturated fatty acids suggests that the very long chain saturated fatty acids isolated in ALD are cytotoxic and are responsible for adrenocortical cell dysfunction in this disease.

Original languageEnglish (US)
Title of host publicationInvestigative and Cell Pathology
Pages353-376
Number of pages24
Volume3
Edition4
StatePublished - 1980
Externally publishedYes

Fingerprint

Adrenal Cortex
Fatty Acids
Lipids
3-Hydroxysteroid Dehydrogenases
Glycerolphosphate Dehydrogenase
Smooth Endoplasmic Reticulum
Rough Endoplasmic Reticulum
Enzymes
Adrenal Glands
Adrenocorticotropic Hormone
Atrophy
Microscopy
Electron Microscopy
Cholesterol
Light

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Powers, J. M., Schaumburg, H. H., Johnson, A. B., & Raine, C. S. (1980). A correlative study of the adrenal cortex in adreno-leukodystrophy: Evidence for a fatal intoxication with very long chain saturated fatty acids. In Investigative and Cell Pathology (4 ed., Vol. 3, pp. 353-376)

A correlative study of the adrenal cortex in adreno-leukodystrophy : Evidence for a fatal intoxication with very long chain saturated fatty acids. / Powers, J. M.; Schaumburg, H. H.; Johnson, A. B.; Raine, C. S.

Investigative and Cell Pathology. Vol. 3 4. ed. 1980. p. 353-376.

Research output: Chapter in Book/Report/Conference proceedingChapter

Powers, JM, Schaumburg, HH, Johnson, AB & Raine, CS 1980, A correlative study of the adrenal cortex in adreno-leukodystrophy: Evidence for a fatal intoxication with very long chain saturated fatty acids. in Investigative and Cell Pathology. 4 edn, vol. 3, pp. 353-376.
Powers JM, Schaumburg HH, Johnson AB, Raine CS. A correlative study of the adrenal cortex in adreno-leukodystrophy: Evidence for a fatal intoxication with very long chain saturated fatty acids. In Investigative and Cell Pathology. 4 ed. Vol. 3. 1980. p. 353-376
Powers, J. M. ; Schaumburg, H. H. ; Johnson, A. B. ; Raine, C. S. / A correlative study of the adrenal cortex in adreno-leukodystrophy : Evidence for a fatal intoxication with very long chain saturated fatty acids. Investigative and Cell Pathology. Vol. 3 4. ed. 1980. pp. 353-376
@inbook{b809761345584f568a221d21580eb294,
title = "A correlative study of the adrenal cortex in adreno-leukodystrophy: Evidence for a fatal intoxication with very long chain saturated fatty acids",
abstract = "Thirty adrenal glands from patients with adreno-leukodystrophy (ALD) have been studied by light microscopy, three by enzyme histochemistry, three by electron microscopy and two by tissue culture. Cytoplasmic ballooning and striations result from proliferation of smooth endoplasmic reticulum and accumulations of lamellar-lipid profiles and clear clefts (crystalloids). Striated adrenocortical cells, the only pathognomonic adrenal lesion in ALD, display cytoplasmic lamellae, decreased amounts of rough endoplasmic reticulum and depression of several enzymes (alpha-glycerophosphate dehydrogenase, 3β-hydroxysteroid dehydrogenase and TPNH diaphorase). The striated cells also demonstrate decreased ability to adapt to changes in microenvironment, both in vivo and in vitro. A blunted response by striated cells to focal peripheral cytolysis leads to cytoplasmic erosion, atrophy and macrovacuoles. ACTH has a pivotal role in the evolution of these lesions. The authors propose that the pathognomonic lamellae of ALD basically represent bilayers or bimolecular leaflets of very long chain saturated fatty acids, while lamellar-lipid profiles and clefts contain cholesterol esterified to these abnormal fatty acids. The similarity of lamellar-lipid profiles of ALD to cytoplasmic lesions induced by long chain saturated fatty acids suggests that the very long chain saturated fatty acids isolated in ALD are cytotoxic and are responsible for adrenocortical cell dysfunction in this disease.",
author = "Powers, {J. M.} and Schaumburg, {H. H.} and Johnson, {A. B.} and Raine, {C. S.}",
year = "1980",
language = "English (US)",
volume = "3",
pages = "353--376",
booktitle = "Investigative and Cell Pathology",
edition = "4",

}

TY - CHAP

T1 - A correlative study of the adrenal cortex in adreno-leukodystrophy

T2 - Evidence for a fatal intoxication with very long chain saturated fatty acids

AU - Powers, J. M.

AU - Schaumburg, H. H.

AU - Johnson, A. B.

AU - Raine, C. S.

PY - 1980

Y1 - 1980

N2 - Thirty adrenal glands from patients with adreno-leukodystrophy (ALD) have been studied by light microscopy, three by enzyme histochemistry, three by electron microscopy and two by tissue culture. Cytoplasmic ballooning and striations result from proliferation of smooth endoplasmic reticulum and accumulations of lamellar-lipid profiles and clear clefts (crystalloids). Striated adrenocortical cells, the only pathognomonic adrenal lesion in ALD, display cytoplasmic lamellae, decreased amounts of rough endoplasmic reticulum and depression of several enzymes (alpha-glycerophosphate dehydrogenase, 3β-hydroxysteroid dehydrogenase and TPNH diaphorase). The striated cells also demonstrate decreased ability to adapt to changes in microenvironment, both in vivo and in vitro. A blunted response by striated cells to focal peripheral cytolysis leads to cytoplasmic erosion, atrophy and macrovacuoles. ACTH has a pivotal role in the evolution of these lesions. The authors propose that the pathognomonic lamellae of ALD basically represent bilayers or bimolecular leaflets of very long chain saturated fatty acids, while lamellar-lipid profiles and clefts contain cholesterol esterified to these abnormal fatty acids. The similarity of lamellar-lipid profiles of ALD to cytoplasmic lesions induced by long chain saturated fatty acids suggests that the very long chain saturated fatty acids isolated in ALD are cytotoxic and are responsible for adrenocortical cell dysfunction in this disease.

AB - Thirty adrenal glands from patients with adreno-leukodystrophy (ALD) have been studied by light microscopy, three by enzyme histochemistry, three by electron microscopy and two by tissue culture. Cytoplasmic ballooning and striations result from proliferation of smooth endoplasmic reticulum and accumulations of lamellar-lipid profiles and clear clefts (crystalloids). Striated adrenocortical cells, the only pathognomonic adrenal lesion in ALD, display cytoplasmic lamellae, decreased amounts of rough endoplasmic reticulum and depression of several enzymes (alpha-glycerophosphate dehydrogenase, 3β-hydroxysteroid dehydrogenase and TPNH diaphorase). The striated cells also demonstrate decreased ability to adapt to changes in microenvironment, both in vivo and in vitro. A blunted response by striated cells to focal peripheral cytolysis leads to cytoplasmic erosion, atrophy and macrovacuoles. ACTH has a pivotal role in the evolution of these lesions. The authors propose that the pathognomonic lamellae of ALD basically represent bilayers or bimolecular leaflets of very long chain saturated fatty acids, while lamellar-lipid profiles and clefts contain cholesterol esterified to these abnormal fatty acids. The similarity of lamellar-lipid profiles of ALD to cytoplasmic lesions induced by long chain saturated fatty acids suggests that the very long chain saturated fatty acids isolated in ALD are cytotoxic and are responsible for adrenocortical cell dysfunction in this disease.

UR - http://www.scopus.com/inward/record.url?scp=0019175034&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0019175034&partnerID=8YFLogxK

M3 - Chapter

VL - 3

SP - 353

EP - 376

BT - Investigative and Cell Pathology

ER -