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

Research output: Contribution to journalArticlepeer-review

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 languageEnglish (US)
Pages (from-to)1223-1229
Number of pages7
JournalBiochemical and Biophysical Research Communications
Volume102
Issue number4
DOIs
StatePublished - Oct 30 1981

ASJC Scopus subject areas

  • Biophysics
  • Biochemistry
  • Molecular Biology
  • Cell Biology

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