Adrenoleukodystrophy: Impaired oxidation of long chain fatty acids in cultured skin fibroblasts and adrenal cortex

Inderjit Singh; Hugo W. Moser; Ann B. Moser; Yasuo Kishimoto

doi:10.1016/S0006-291X(81)80142-8

Adrenoleukodystrophy: Impaired oxidation of long chain fatty acids in cultured skin fibroblasts and adrenal cortex

Inderjit Singh, Hugo W. Moser, Ann B. Moser, Yasuo Kishimoto

Kennedy Krieger Institute

Research output: Contribution to journal › Article › peer-review

100 Scopus citations

Abstract

CO₂ production from [1-¹⁴C] fatty acids was studied with cultured skin fibroblasts and adrenal gland from adrenoleukodystrophy (ALD) patients. The CO₂ production from lignoceric acid in 11 and from hexacosanoic acid in 4 ALD cultured skin fibroblasts cultures was reduced to 8.2 ± 9 and 14 ± 8% of control, respectively. The adrenal studies compared a biopsy specimen from a 7 year old boy with ALD and 6 control adrenals from renal transplant donors. CO₂ production from oleic acid was reduced to less than 10% of control, while cholesterol esterase and cholesterol esterifying enzyme activities were similar to the control.

Original language	English (US)
Pages (from-to)	1223-1229
Number of pages	7
Journal	Biochemical and Biophysical Research Communications
Volume	102
Issue number	4
DOIs	https://doi.org/10.1016/S0006-291X(81)80142-8
State	Published - Oct 30 1981

ASJC Scopus subject areas

Biophysics
Biochemistry
Molecular Biology
Cell Biology

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@article{e738fe039a174bcdae30f9f89869635f,

title = "Adrenoleukodystrophy: Impaired oxidation of long chain fatty acids in cultured skin fibroblasts and adrenal cortex",

abstract = "CO2 production from [1-14C] fatty acids was studied with cultured skin fibroblasts and adrenal gland from adrenoleukodystrophy (ALD) patients. The CO2 production from lignoceric acid in 11 and from hexacosanoic acid in 4 ALD cultured skin fibroblasts cultures was reduced to 8.2 ± 9 and 14 ± 8% of control, respectively. The adrenal studies compared a biopsy specimen from a 7 year old boy with ALD and 6 control adrenals from renal transplant donors. CO2 production from oleic acid was reduced to less than 10% of control, while cholesterol esterase and cholesterol esterifying enzyme activities were similar to the control.",

author = "Inderjit Singh and Moser, {Hugo W.} and Moser, {Ann B.} and Yasuo Kishimoto",

note = "Funding Information: ACKNOWLEDGEMENTS: We thank Dr. Eduardo Perusquio Ortego for making available the ALD adrenal biopsy tissues, Dr. James M. Powers for morphological studies, Ms. Jo Lesley and Dr. Melville G. Williams for their help in obtaining the control adrenal tissues, and Ms. Karen Frayer and Surinder K. Khangoora for expert technical assistance. This work was supported in part by Grants NS 13513 and HD 10981, NS 13569 from the United States Public Health Service and BNS-79-23017 from National Science Foundation. Copyright: Copyright 2014 Elsevier B.V., All rights reserved.",

year = "1981",

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day = "30",

doi = "10.1016/S0006-291X(81)80142-8",

language = "English (US)",

volume = "102",

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T1 - Adrenoleukodystrophy

T2 - Impaired oxidation of long chain fatty acids in cultured skin fibroblasts and adrenal cortex

AU - Singh, Inderjit

AU - Moser, Hugo W.

AU - Moser, Ann B.

AU - Kishimoto, Yasuo

N1 - Funding Information: ACKNOWLEDGEMENTS: We thank Dr. Eduardo Perusquio Ortego for making available the ALD adrenal biopsy tissues, Dr. James M. Powers for morphological studies, Ms. Jo Lesley and Dr. Melville G. Williams for their help in obtaining the control adrenal tissues, and Ms. Karen Frayer and Surinder K. Khangoora for expert technical assistance. This work was supported in part by Grants NS 13513 and HD 10981, NS 13569 from the United States Public Health Service and BNS-79-23017 from National Science Foundation. Copyright: Copyright 2014 Elsevier B.V., All rights reserved.

PY - 1981/10/30

Y1 - 1981/10/30

N2 - CO2 production from [1-14C] fatty acids was studied with cultured skin fibroblasts and adrenal gland from adrenoleukodystrophy (ALD) patients. The CO2 production from lignoceric acid in 11 and from hexacosanoic acid in 4 ALD cultured skin fibroblasts cultures was reduced to 8.2 ± 9 and 14 ± 8% of control, respectively. The adrenal studies compared a biopsy specimen from a 7 year old boy with ALD and 6 control adrenals from renal transplant donors. CO2 production from oleic acid was reduced to less than 10% of control, while cholesterol esterase and cholesterol esterifying enzyme activities were similar to the control.

AB - CO2 production from [1-14C] fatty acids was studied with cultured skin fibroblasts and adrenal gland from adrenoleukodystrophy (ALD) patients. The CO2 production from lignoceric acid in 11 and from hexacosanoic acid in 4 ALD cultured skin fibroblasts cultures was reduced to 8.2 ± 9 and 14 ± 8% of control, respectively. The adrenal studies compared a biopsy specimen from a 7 year old boy with ALD and 6 control adrenals from renal transplant donors. CO2 production from oleic acid was reduced to less than 10% of control, while cholesterol esterase and cholesterol esterifying enzyme activities were similar to the control.

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Adrenoleukodystrophy: Impaired oxidation of long chain fatty acids in cultured skin fibroblasts and adrenal cortex

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