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Bikunin (Bkn) isoforms are serum chondroitin sulfate (CS) proteoglycans synthetized by the liver. They include two light Bkn forms, i.e. the core free Bkn protein and the Bkn linked to the CS chain (UTI) and two heavy Bkn forms, i.e. PαI and ITI, corresponding to UTI esterified by one or two heavy chains (HC) glycoproteins, respectively. We previously showed that the Western-blot analysis of the light Bkn forms allows to rapidly and easily detect the linkeropathies diseases corresponding to inherited defects in enzymes involved in the synthesis of the initial common tetrasaccharide linker of proteoglycans.

We also showed alterations in the ester linkages of HC in several subtypes of CDG.

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