Nature Communications (Jan 2020)

Crystal structures of fukutin-related protein (FKRP), a ribitol-phosphate transferase related to muscular dystrophy

  • Naoyuki Kuwabara,
  • Rieko Imae,
  • Hiroshi Manya,
  • Tomohiro Tanaka,
  • Mamoru Mizuno,
  • Hiroki Tsumoto,
  • Motoi Kanagawa,
  • Kazuhiro Kobayashi,
  • Tatsushi Toda,
  • Toshiya Senda,
  • Tamao Endo,
  • Ryuichi Kato

DOI
https://doi.org/10.1038/s41467-019-14220-z
Journal volume & issue
Vol. 11, no. 1
pp. 1 – 11

Abstract

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Fukutin-related protein (FKRP) catalyses the addition of ribitol-phosphate (RboP) to the O-mannosyl glycan of α-dystroglycan and mutations in FKRP cause dystroglycanopathy. Here the authors provide insights into its oligomerization and recognition of the substrates, CDP-Rbo and the RboP-(phospho-)core M3 glycan, by determining the crystal structures of human FKRP.