Ribitol-5-phosphate xylosyltransferase 1

Definition:

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 (Probable). Participates 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 (Probable).

References:

[1]. Hirokazu Yagi, et al. Direct Mapping of Additional Modifications on Phosphorylated O-glycans of α-Dystroglycan by Mass Spectrometry Analysis in Conjunction with Knocking Out of Causative Genes for Dystroglycanopathy. Mol Cell Proteomics. 2016 Nov;15(11):3424-3434. [Content Brief]

[2]. Jeremy L Praissman, et al. The functional O-mannose glycan on α-dystroglycan contains a phospho-ribitol primed for matriglycan addition. Elife. 2016 Apr 29;5:e14473. [Content Brief]

[3]. Tobias Willer, et al. The glucuronyltransferase B4GAT1 is required for initiation of LARGE-mediated α-dystroglycan functional glycosylation. Elife. 2014 Oct 3;3:e03941. [Content Brief]

[4]. Ryuta Nishihara, et al. Cell endogenous activities of fukutin and FKRP coexist with the ribitol xylosyltransferase, TMEM5. Biochem Biophys Res Commun. 2018 Mar 18;497(4):1025-1030. [Content Brief]

[5]. Hiroshi Manya, et al. The Muscular Dystrophy Gene TMEM5 Encodes a Ribitol β1,4-Xylosyltransferase Required for the Functional Glycosylation of Dystroglycan. J Biol Chem. 2016 Nov 18;291(47):24618-24627. [Content Brief]

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