التفاصيل البيبلوغرافية
| العنوان: |
Case study: monitoring of Glc4 tetrasaccharide in the urine of Pompe patients, use of MALDI-TOF MS, and 1H NMR. |
| المؤلفون: |
Pakanová, Zuzana, Matulová, Mária, Uhliariková, Iveta, Behúlová, Darina, Šalingová, Anna, Hlavatá, Anna, Juríčková, Katarína, Nemčovič, Marek, Pätoprstý, Vladimír, Mucha, Ján |
| المصدر: |
Chemical Papers; Mar2019, Vol. 73 Issue 3, p701-711, 11p |
| مستخلص: |
Due to the lack of the acid 1,4-α-glucosidase, the urine of Pompe patients contains increased levels of specific glucose tetrasaccharide αGlc(1 → 6)αGlc(1 → 4)αGlc(1 → 4)Glc (Glc4), which was approved as a non-invasive biomarker of glycogen accumulation in the muscles of Pompe patients. MALDI-TOF mass spectrometry and 1H NMR spectroscopy were used for Glc4 detection in the urine samples of four Pompe patients with infantile, juvenile, and adult forms of the disease, before and during, or before and after, enzyme replacement therapy. The increased sensitivity and decreased urine volume required for Glc4 detection by MALDI-TOF mass spectrometry was obtained by specific derivatization directly on the MALDI plate by 3-aminoquinoline. This method is a suitable and fast method for Pompe screening. 1H NMR spectra, used for precise Glc4 quantifications, were proposed for the enzyme replacement therapy efficiency monitoring. Besides the clinical features and the biochemical parameters, knowledge about the evolution of the Glc4 levels over time during the administration of the therapy could help monitoring of the patient outcome as part of the standard protocol. [ABSTRACT FROM AUTHOR] |
|
Copyright of Chemical Papers is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.) |
| قاعدة البيانات: |
Complementary Index |
Find this article in full text from Springer Verlag. 
Full Text Finder