Carnitine palmitoyltransferase 1A deficiency
Jan. 02, 2024
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Schematic representation of the glycosylation pathways involving galactose
Galactose is metabolized into UDP-Gal, which is used as a precursor for the glycosylation of different macromolecules. UDP-Gal can be used as a building block for synthesis of N-glycans and O-glycans. Moreover, UDP-Gal can be used to synthesize keratan sulfate, a glycosaminoglycan that, along with N- and O-glycans, is an essential component of several extracellular proteoglycans. Additionally, UDP-Gal is used to glycosylate collagens. Lastly, UDP-Gal can be used to glycosylate lipids alone or in combination with other monosaccharides, generating glycolipids. Among glycolipids, several types of glyceroglycolipids, sulfoglycolipids, and glycerolipids contain galactose in their sugar moieties. (Source: Conte F, van Buuringen N, Voermans NC, Lefeber DJ. Galactose in human metabolism, glycosylation, and congenital metabolic diseases: time for a closer look. Biochim Biophys Acta Gen Subj 2021;1865[8]:129898. CC BY License, http://creativecommons.org/licenses/by/4.0.)