Synthesis of Mevalonate Pathway Lipids in Fibroblasts from Zellweger and X-linked ALD Patients
作者:
EEVA-LIISA APPELKVIST,
NIKOLAOS VENIZELOS,
YIYI ZHANG,
INGELA PARMRYD,
LARS HAGENFELDT,
GUSTAV DALLNER,
期刊:
Pediatric Research
(OVID Available online 1999)
卷期:
Volume 46,
issue 3
页码: 345-350
ISSN:0031-3998
年代: 1999
出版商: OVID
数据来源: OVID
摘要:
Fibroblasts were cultured to determine the involvement of peroxisomes in cholesterol and dolichol synthesis. For this purpose, the behavior of cells from patients with Zellweger syndrome, with X-linked adrenoleukodystrophy, and from nondiseased control subjects was studied. Cells both after pretreatment with mevinolin and without pretreatment were incubated in a medium containing [3H]-mevalonate. In fibroblasts from patients with peroxisomal defects, the cholesterol content and mevalonate incorporation into cholesterol were decreased by 10-20% in comparison with control cells. Mevinolin pretreatment decreased the incorporation rate of [3H]-mevalonate into cholesterol but increased the labeling of ubiquinone and dolichol both in diseased and control cells. Squalene synthase activity was unchanged, whereas the activity of farnesyl-pyrophosphate synthase was increased in the diseased states. The results show that in patients with peroxisomal deficiency neither the amount nor the rate of synthesis of cholesterol and dolichol is reduced to any greater extent.
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