Glycosaminoglycans analysis in blood and urine of patients with mucopolysaccharidosis

Molecular Genetics and Metabolism
Shaukat A KhanShunji Tomatsu

Abstract

To explore the correlation between glycosaminoglycan (GAG) levels and mucopolysaccharidosis (MPS) type, we have evaluated the GAG levels in blood of MPS II, III, IVA, and IVB and urine of MPS IVA, IVB, and VI by tandem mass spectrometry. Dermatan sulfate (DS), heparan sulfate (HS), keratan sulfate (KS; mono-sulfated KS, di-sulfated KS), and the ratio of di-sulfated KS in total KS were measured. Patients with untreated MPS II had higher levels of DS and HS in blood while untreated MPS III had higher levels of HS in blood than age-matched controls. Untreated MPS IVA had higher levels of KS in blood and urine than age-matched controls. The ratio of blood di-sulfated KS/total KS in untreated MPS IVA was constant and higher than that in controls for children up to 10 years of age. The ratio of urine di-sulfated KS/total KS in untreated MPS IVA was also higher than that in age-matched controls, but the ratio in untreated MPS IVB was lower than controls. ERT reduced blood DS and HS in MPS II, and urine KS in MPS IVA patients, although GAGs levels remained higher than the observed in age-matched controls. ERT did not change blood KS levels in MPS IVA. MPS VI under ERT still had an elevation of urine DS level compared to age-matched con...Continue Reading

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Citations

Aug 29, 2019·Journal of Human Genetics·Hui Hsuan ChenShunji Tomatsu
Feb 20, 2020·International Journal of Molecular Sciences·Francesca D'AvanzoRosella Tomanin
Jun 15, 2019·Orphanet Journal of Rare Diseases·Mehmet Umut AkyolUNKNOWN MPS Consensus Programme Co-Chairs
Apr 29, 2020·International Journal of Molecular Sciences·Rossella Parini, Federica Deodato
Mar 20, 2020·International Journal of Molecular Sciences·Tsubasa OguniHironori Kobayashi
Apr 26, 2019·Journal of Human Genetics·Caitlin DohertyShunji Tomatsu
Aug 28, 2019·International Journal of Molecular Sciences·Kazuki Sawamoto, Shunji Tomatsu
Feb 28, 2020·International Journal of Molecular Sciences·Kazuki SawamotoShunji Tomatsu
Feb 20, 2020·JIMD Reports·Iman S AbumansourSylvia Stockler-Ipsiroglu
Dec 4, 2020·International Journal of Molecular Sciences·Nataliya YuskivSylvia Stockler-Ipsiroglu
Jan 1, 2021·International Journal of Molecular Sciences·Víctor J ÁlvarezMaría L Couce
Jun 25, 2020·Molecular Therapy. Methods & Clinical Development·Kazuki SawamotoShunji Tomatsu
Mar 4, 2021·Expert Opinion on Biological Therapy·Azam SafaryYadollah Omidi
Jul 15, 2021·Nature Communications·Sourav K BoseWilliam H Peranteau

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