Catalyzes the first step in the biosynthesis of chondroitin sulfate and dermatan sulfate proteoglycans, such as DCN. Transfers D-xylose from UDP-D-xylose to specific serine residues of the core protein ( , ). Required for normal embryonic and postnatal skeleton development, especially of the long bones (, ). Required for normal maturation of chondrocytes during bone development, and normal onset of ossification (By similarity).
Subcellular locations: Golgi apparatus membrane, Secreted
Detected predominantly in the Golgi apparatus.
Widely expressed. Expressed at higher level in placenta, kidney and pancreas. Weakly expressed in skeletal muscle.