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• W4220713058 endingPage "678" @default.
• W4220713058 startingPage "678" @default.
• W4220713058 abstract "Mucopolysaccharidosis, type IIIB (MPS IIIB) is a rare disease caused by mutations in the N-alpha-acetylglucosaminidase (NAGLU) gene resulting in decreased or absent enzyme activity. On the cellular level, the disorder is characterized by the massive lysosomal storage of heparan sulfate (HS)-one species of glycosaminoglycans. HS is a sulfur-rich macromolecule, and its accumulation should affect the turnover of total sulfur in cells; according to the studies presented here, it, indeed, does. The lysosomal degradation of HS in cells produces monosaccharides and inorganic sulfate (SO42-). Sulfate is a product of L-cysteine metabolism, and any disruption of its levels affects the entire L-cysteine catabolism pathway, which was first reported in 2019. It is known that L-cysteine level is elevated in cells with the Naglu-/- gene mutation and in selected tissues of individuals with MPS IIIB. The level of glutathione and the Naglu-/- cells' antioxidant potential are significantly reduced, as well as the activity of 3-mercaptopyruvate sulfurtransferase (MPST, EC 2.8.1.2) and the level of sulfane sulfur-containing compounds. The direct reason is not yet known. This paper attempts to identify some of cause-and-effect correlations that may lead to this condition and identifies research directions that should be explored." @default.
• W4220713058 created "2022-04-03" @default.
• W4220713058 creator A5000361602 @default.
• W4220713058 creator A5057282247 @default.
• W4220713058 creator A5081543883 @default.
• W4220713058 date "2022-03-30" @default.
• W4220713058 modified "2023-09-25" @default.
• W4220713058 title "Heparan Sulfate, Mucopolysaccharidosis IIIB and Sulfur Metabolism Disorders" @default.
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