鉴定中链酰基辅酶A脱氢酶缺乏症患者尿液中存在的新型中链酰基肉碱。
Identification of new medium-chain acylcarnitines present in urine of a patient with medium-chain acyl-CoA dehydrogenase deficiency.
作者信息
Libert R, Van Hoof F, Thillaye M, Vincent M F, Nassogne M C, Stroobant V, de Hoffmann E, Schanck A
机构信息
Département de Biologie Clinique, Cliniques Universitaires St Luc, Brussels, Belgium.
出版信息
J Inherit Metab Dis. 1999 Feb;22(1):9-18. doi: 10.1023/a:1005482828392.
Previously undescribed medium-chain acylcarnitines were identified in a urine sample from a patient with medium-chain acyl-CoA dehydrogenase deficiency. These are the 4-methylvaleryl, 4- and 5-methylhexanoyl, 6-methylheptanoyl-, 6-methyloctanoyl-, 4,5-dimethylhexanoyl- and 4,7-decadienoylcarnitines. Their chemical structures were obtained by gas chromatographymass spectrometry analysis of their fatty acid moieties as picolinyl esters.
在一名患有中链酰基辅酶A脱氢酶缺乏症患者的尿液样本中，鉴定出了此前未描述过的中链酰基肉碱。这些是4-甲基戊酰基、4-和5-甲基己酰基、6-甲基庚酰基、6-甲基辛酰基、4,5-二甲基己酰基和4,7-癸二烯酰基肉碱。通过对其脂肪酸部分作为吡啶基酯进行气相色谱-质谱分析，获得了它们的化学结构。
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