Mills Kevin, Eaton Simon, Ledger Victoria, Young Elisabeth, Winchester Bryan
Biochemistry, Endocrinology and Metabolism Unit, Institute of Child Health, University College London, 30 Guilford Street, London WC1N 1EH, UK.
Rapid Commun Mass Spectrom. 2005;19(12):1739-48. doi: 10.1002/rcm.1977.
Novel internal standards have been synthesised for the quantitative determination by tandem mass spectrometry (MS/MS) of the sphingolipids that accumulate in lysosomal storage diseases. The [d4]C16- and [d47]C24-isoforms of galactosylceramide (CMH), lactosylceramide (CDH), globotriaosylceramide (CTH), cerebroside sulphate, sphingomyelin and G(M1)-, G(M2)- and G(M3)-gangliosides were synthesised by the reaction of their lyso-forms with the acid chlorides of hexadecanoic 5,5,6,6-d4 acid ([d4]-palmitic acid) and tetracosanoic-d47 acid ([d47]-lignoceric acid), respectively. The acid chlorides were formed using oxalyl chloride. The structures of the internal standards were confirmed by MS/MS. The fragmentation pattern of each novel compound was similar to that of the corresponding natural form of the sphingolipid, making it a good internal standard for the quantitative determination of the natural sphingolipid by ESI-MS/MS. Characteristic product ions were identified for each compound.
已合成新型内标物，用于通过串联质谱（MS/MS）对溶酶体贮积病中蓄积的鞘脂进行定量测定。半乳糖神经酰胺（CMH）、乳糖神经酰胺（CDH）、球三糖神经酰胺（CTH）、硫酸脑苷脂、鞘磷脂以及G(M1)-、G(M2)-和G(M3)-神经节苷脂的[d4]C16和[d47]C24异构体分别通过其溶血形式与十六烷酸5,5,6,6-d4酸（[d4]-棕榈酸）和二十四烷酸-d47酸（[d47]-木蜡酸）的酰氯反应合成。酰氯使用草酰氯制备。内标物的结构通过MS/MS确证。每种新型化合物的碎裂模式与相应鞘脂天然形式的碎裂模式相似，使其成为通过电喷雾电离串联质谱（ESI-MS/MS）对天然鞘脂进行定量测定的良好内标物。已为每种化合物鉴定出特征性产物离子。
