کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
10536271 | 962056 | 2005 | 17 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Profiling oligosaccharidurias by electrospray tandem mass spectrometry: Quantifying reducing oligosaccharides
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کلمات کلیدی
Electrospray ionization-tandem mass spectrometryGM2 gangliosidosis1-phenyl-3-methyl-5-pyrazoloneGM1 gangliosidosisα-Mannosidosis - α-منوسیتوزیسLysosomal storage disorder - اختلال ذخیره سازی لیزوزومیPompe - تلمبهSialidosis - سیلیدوزFucosidosis - فوکوسیدوزMetachromatic leukodystrophy - لکوودیستروفی متاکروماتیکGalactosialidosis - گالاکتوسیالیدوزGaucher - گوچه
موضوعات مرتبط
مهندسی و علوم پایه
شیمی
شیمی آنالیزی یا شیمی تجزیه
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
A method to semiquantify urinary oligosaccharides from patients suffering from oligosaccharidurias is presented. 1-Phenyl-3-methyl-5-pyrazolone has been used to derivatize urinary oligosaccharides prior to analysis by electrospray ionization-tandem mass spectrometry (ESI-MS/MS). Disease-specific oligosaccharides were identified for several oligosaccharidurias, including GM1 gangliosidosis, GM2 gangliosidosis, sialic acid storage disease, sialidase/neuraminidase deficiency, galactosialidosis, I-cell disease, fucosidosis, Pompe and Gaucher diseases, and α-mannosidosis. The oligosaccharides were referenced against the internal standard, methyl lactose, to produce ratios for comparison with control samples. Elevations in specific urinary oligosaccharides were indicative of lysosomal disease and the defective catabolic enzyme. This method has been adapted to enable assay of large sample numbers and could readily be extended to other oligosaccharidurias and to monitor oligosaccharide levels in patients receiving treatment. It also has immediate potential for incorporation into a newborn screening program.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Analytical Biochemistry - Volume 345, Issue 1, 1 October 2005, Pages 30-46
Journal: Analytical Biochemistry - Volume 345, Issue 1, 1 October 2005, Pages 30-46
نویسندگان
Steven L. Ramsay, Peter J. Meikle, John J. Hopwood, Peter R. Clements,