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Sökning: WFRF:(Venizelos N.) > Synthesis of mevalo...

LIBRIS Formathandbok  (Information om MARC21)
FältnamnIndikatorerMetadata
00002489naa a2200349 4500
001oai:DiVA.org:uu-193093
003SwePub
008130129s1999 | |||||||||||000 ||eng|
009oai:prod.swepub.kib.ki.se:1928456
024a https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-1930932 URI
024a https://doi.org/10.1203/00006450-199909000-000172 DOI
024a http://kipublications.ki.se/Default.aspx?queryparsed=id:19284562 URI
040 a (SwePub)uud (SwePub)ki
041 a engb eng
042 9 SwePub
072 7a ref2 swepub-contenttype
072 7a art2 swepub-publicationtype
100a Appelkvist, E L4 aut
2451 0a Synthesis of mevalonate pathway lipids in fibroblasts from Zellweger and X-linked ALD patients.
264 1b Springer Science and Business Media LLC,c 1999
338 a print2 rdacarrier
520 a 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.
700a Venizelos, N4 aut
700a Zhang, Y4 aut
700a Parmryd, I4 aut
700a Hagenfeldt, L4 aut
700a Dallner, G4 aut
773t Pediatric Researchd : Springer Science and Business Media LLCg 46:3, s. 345-50q 46:3<345-50x 0031-3998x 1530-0447
856u https://www.nature.com/articles/pr19992823.pdf
8564 8u https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-193093
8564 8u https://doi.org/10.1203/00006450-199909000-00017
8564 8u http://kipublications.ki.se/Default.aspx?queryparsed=id:1928456

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