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Updated: May 29, 2026

Metabolic Glycoengineering of Sialic Acid Using N-acyl-modified Mannosamines
Published on: November 25, 2017
[Mucopolysaccharidoses--biochemical mechanisms of diseases and therapeutic possibilities]
Anna Kloska1, Anna Tylki-Szymańska, Grzegorz Wegrzyn
1Katedra Biologii Molekularnej, Wydzial Biologii, Uniwersytet Gdański, Gdańsk.
Abstract:
Mucopolysaccharidoses (MPS) are inherited metabolic diseases from the group of lysosomal storage disorders (LSD). They are caused by genetic defects resulting in the absence or severe deficiency in one of lysosmal hydrolases involved in degradation of glycosaminoglycans (GAG). Partially degraded GAGs are accumulated in lysosomes, causing dysfunction of cells, tissues and organs. Last years did bring some breakthrough discoveries, which were important to understand biochemical mechanisms of MPS appearance and course, as well as to develop therapeutic procedures for these inherited metabolic disorders.
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