FEATURES

FAMILY

DESCRIPTION

Also known as RXLT1_HUMAN, RXYLT1, TMEM5. UDP-xylosyltransferase involved in the biosynthesis of the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1), which is required for binding laminin G-like domain-containing extracellular proteins with high affinity (PubMed:25279699, PubMed:27601598, PubMed:27733679) (Probable). Acts as a UDP-D-xylose:ribitol-5-phosphate beta1, 4-xylosyltransferase, which catalyzes the transfer of UDP-D-xylose to ribitol 5-phosphate (Rbo5P) to form the Xylbeta1-4Rbo5P linkage on O-mannosyl glycan (PubMed:27733679, PubMed:29477842) (Probable). Forms a complex composed of FKTN/fukutin, FKRP and RXYLT1/TMEM5.

EXTERNAL LINKS

RXYLT1

Ribitol-5-phosphate xylosyltransferase 1

RXYLT1

Molecular Synopsis