uu.seUppsala University Publications
Change search
ReferencesLink to record
Permanent link

Direct link
Synthesis of mevalonate pathway lipids in fibroblasts from Zellweger and X-linked ALD patients.
Show others and affiliations
1999 (English)In: Pediatric Research, ISSN 0031-3998, E-ISSN 1530-0447, Vol. 46, no 3, 345-50 p.Article in journal (Refereed) Published
Abstract [en]

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.

Place, publisher, year, edition, pages
1999. Vol. 46, no 3, 345-50 p.
National Category
Medical and Health Sciences
URN: urn:nbn:se:uu:diva-193093DOI: 10.1203/00006450-199909000-00017PubMedID: 10473053OAI: oai:DiVA.org:uu-193093DiVA: diva2:601314
Available from: 2013-01-29 Created: 2013-01-29 Last updated: 2013-01-29

Open Access in DiVA

No full text

Other links

Publisher's full textPubMed

Search in DiVA

By author/editor
Parmryd, I
In the same journal
Pediatric Research
Medical and Health Sciences

Search outside of DiVA

GoogleGoogle Scholar
The number of downloads is the sum of all downloads of full texts. It may include eg previous versions that are now no longer available

Altmetric score

Total: 225 hits
ReferencesLink to record
Permanent link

Direct link