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放射性标记的半乳糖基-(α1 至 4)-半乳糖基-(β1 至 4)-葡萄糖神经酰胺在体外被人血清脂蛋白摄取的情况。

Uptake of radiolabeled galactosyl-(alpha1 goes to 4)-galactosyl-(beta1 goes to 4)-glucosylceramide by human serum lipoproteins in vitro.

作者信息

Clarke J T, Stoltz J M

出版信息

Biochim Biophys Acta. 1976 Jul 20;441(1):165-9. doi: 10.1016/0005-2760(76)90291-5.

DOI:10.1016/0005-2760(76)90291-5
PMID:182248
Abstract

Human serum was exposed to various amounts of [6-3H] galactosyl-(alpha1 goes to 4)-galactosyl-(beta1 goes to 4)-glucosylceramide under standardized conditions in vitro, and the uptake of the lipid by serum lipoproteins was determined. Of the bound glycolipid, 2% was isolated with very low density, 24% with low density-, 47% with high density lipoproteins and 27% with the ultracentrifugal residue. The distribution was different from the distribution of endogenous galactosyl-galactosylglucosylceramide, indicating that the glycolipid is probably an integral part of the lipoprotein complexes in vivo.

摘要

在体外标准化条件下,将人血清暴露于不同量的[6-³H]半乳糖基-(α1→4)-半乳糖基-(β1→4)-葡萄糖神经酰胺中,然后测定血清脂蛋白对该脂质的摄取。在结合的糖脂中,2%与极低密度脂蛋白一起分离出来,24%与低密度脂蛋白一起,47%与高密度脂蛋白一起,27%与超速离心残渣一起。这种分布与内源性半乳糖基-半乳糖基葡萄糖神经酰胺的分布不同,表明该糖脂可能是体内脂蛋白复合物的一个组成部分。

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Uptake of radiolabeled galactosyl-(alpha1 goes to 4)-galactosyl-(beta1 goes to 4)-glucosylceramide by human serum lipoproteins in vitro.放射性标记的半乳糖基-(α1 至 4)-半乳糖基-(β1 至 4)-葡萄糖神经酰胺在体外被人血清脂蛋白摄取的情况。
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2
Infusion of alpha-galactosidase A reduces tissue globotriaosylceramide storage in patients with Fabry disease.输注α-半乳糖苷酶A可减少法布里病患者组织中球三糖神经酰胺的蓄积。
Proc Natl Acad Sci U S A. 2000 Jan 4;97(1):365-70. doi: 10.1073/pnas.97.1.365.
3
Enzyme therapy in Fabry disease: differential in vivo plasma clearance and metabolic effectiveness of plasma and splenic alpha-galactosidase A isozymes.
Proc Natl Acad Sci U S A. 1979 Oct;76(10):5326-30. doi: 10.1073/pnas.76.10.5326.