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- Appelkvist, E L, et al.
(författare)
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Synthesis of mevalonate pathway lipids in fibroblasts from Zellweger and X-linked ALD patients.
- 1999
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Ingår i: Pediatric Research. - : Springer Science and Business Media LLC. - 0031-3998 .- 1530-0447. ; 46:3, s. 345-50
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Tidskriftsartikel (refereegranskat)abstract
- 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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| 4. |
- Thorstensen, O, et al.
(författare)
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MnDPDP enhancement in rabbit liver after intravenous bolus injection and slow infusion
- 1997
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Ingår i: Acta radiologica (Stockholm, Sweden : 1987). - : SAGE Publications. - 0284-1851 .- 1600-0455. ; 38:44 Pt 2, s. 717-723
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Tidskriftsartikel (refereegranskat)abstract
- Purpose: To investigate the MR-enhancing effect of mangafodipir trisodium (MnDPDP, Teslascan) in the rabbit liver in relation to dose, mode of administration and imaging window. Material and Methods: MnDPDP was administered to 18 rabbits at a dose of 10 μmol/kg or 20 μmol/kg, as a bolus injection or infusion. MR imaging of the liver was performed at different time intervals. Results: Peak liver enhancement was typically observed 10–30 min after administration and the enhancement declined with a half-time of about 5 h. This pattern was observed in all sequences (SE 400/15, FLASH, and SE 132/10), with both doses and with both rates of administration. The peak enhancement was greater, though not significantly so after 20 μmol/kg than after 10 μmol/kg. A higher relative peak signal was observed with SE 132/10 than with FLASH or SE 400/15. Conclusion: A good liver imaging result was obtained after a dose of 10 μmol/kg, either bolus or infusion, 10–30 min post-contrast with heavily T1-weighted sequences.
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