CHST3A gene on chromosome 10q22.1 that encodes a sulfotransferase which transfers sulfate to chondroitin, yielding chondroitin sulfate, a predominant proteoglycan of cartilage, cell surfaces and extracellular matrices. CHST3 can also transfer sulfate to keratan, another glycosaminoglycan, and to sialyl N-acetyllactosamine oligosaccharides. It may play a role in maintaining naive T cells in the spleen.
Defects in CHST3 are a cause of spondyloepiphyseal dysplasia with congenital joint dislocations (SEDC-JD).
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