溶酶体贮积症患者血浆中的鞘脂激活蛋白A、B、C和D。
Saposins A, B, C, and D in plasma of patients with lysosomal storage disorders.
作者信息
Chang M H, Bindloss C A, Grabowski G A, Qi X, Winchester B, Hopwood J J, Meikle P J
机构信息
Lysosomal Diseases Research Unit, Department of Chemical Pathology, Women's and Children's Hospital, 72 King William Road, North Adelaide, South Australia 5006, Australia.
出版信息
Clin Chem. 2000 Feb;46(2):167-74.
BACKGROUND
Early diagnosis of lysosomal storage disorders (LSDs), before the onset of irreversible pathology, will be critical for maximum efficacy of many current and proposed therapies. To search for potential markers of LSDs, we measured saposins A, B, C, and D in patients with these disorders.
METHODS
Four time-delayed fluorescence immunoquantification assays were used to measure each of the saposins in plasma from 111 unaffected individuals and 334 LSD-affected individuals, representing 28 different disorders.
RESULTS
Saposin A was increased above the 95th centile of the control population in 59% of LSD patients; saposins B, C, and D were increased in 25%, 61%, and 57%, respectively. Saposins were increased in patients from several LSD groups that in previous studies did not show an increase of lysosome-associated membrane protein-1 (LAMP-1).
CONCLUSION
Saposins may be useful markers for LSDs when used in conjunction with LAMP-1.
背景
在不可逆病理状态出现之前对溶酶体贮积症（LSDs）进行早期诊断，对于许多现有和提议的治疗方法的最大疗效至关重要。为了寻找LSDs的潜在标志物，我们检测了这些疾病患者体内的鞘脂激活蛋白A、B、C和D。
方法
采用四种时间分辨荧光免疫定量分析法，检测了111名未受影响个体和334名LSDs患者血浆中的每种鞘脂激活蛋白，这些患者代表了28种不同的疾病。
结果
59%的LSDs患者的鞘脂激活蛋白A水平高于对照人群的第95百分位数；鞘脂激活蛋白B、C和D水平升高的患者分别占25%、61%和57%。在先前研究中未显示溶酶体相关膜蛋白-1（LAMP-1）升高的几个LSDs组的患者中，鞘脂激活蛋白水平升高。
结论
鞘脂激活蛋白与LAMP-1联合使用时，可能是LSDs的有用标志物。
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