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Diagram of the linkeropathy genes and their associated syndromes. Proteoglycans are highly glycosylated proteins with covalently attached glycosaminoglycan (GAG) chain. The GAG is attached via a tetrasaccharide bridge (indicated by the four green ovals) to a serine (Ser) residue in the core protein. Human disorders caused by mutations affected each step of tetrasaccharide synthesis.
