Article
3D structural analysis of protein O-mannosyl kinase, POMK, a causative gene product of dystroglycanopathy.
Genes to cells : devoted to molecular & cellular mechanisms - 1 Apr 2017
Nagae Masamichi, Mishra Sushil K, Neyazaki Makiko, Oi Rika, Ikeda Akemi, Matsugaki Naohiro, Akashi Satoko, Manya Hiroshi, Mizuno Mamoru, Yagi Hirokazu, Kato Koichi, Senda Toshiya, Endo Tamao, Nogi Terukazu, Yamaguchi Yoshiki
Abstract excerpt
Orchestration of the multiple enzymes engaged in O-mannose glycan synthesis provides a matriglycan on α-dystroglycan (α-DG) which attracts extracellular matrix (ECM) proteins such as laminin. Aberrant O-mannosylation of α-DG leads to severe congenital muscular dystrophies due to detachment of ECM proteins from the basal membrane. Phosphorylation at C6-position of O-mannose catalyzed by protein O-mannosyl kinase...
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