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)|
|Number of pages||7|
|Journal||Biochemical and Biophysical Research Communications|
|State||Published - Oct 30 1981|
ASJC Scopus subject areas
- Molecular Biology
- Cell Biology