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Reduced proliferative activity of primary POMGnT1-null myoblasts in vitro.

Protein O-linked mannose beta1,2-N-acetylglucosaminyltransferase 1 (POMGnT1) is an enzyme that transfers N-acetylglucosamine to O-mannose of glycoproteins. Mutations of the POMGnT1 gene cause muscle-eye-brain (MEB) disease. To obtain a better understanding of the pathogenesis of MEB disease, we mutated the POMGnT1 gene in mice using a targeting technique. The mutant muscle showed aberrant glycosylation of alpha-DG, and alpha-DG from mutant muscle failed to bind laminin in a binding assay. POMGnT1(-/-) muscle showed minimal pathological changes with very low-serum creatine kinase levels, and had normally formed muscle basal lamina, but showed reduced muscle mass, reduced numbers of muscle fibers, and impaired muscle regeneration. Importantly, POMGnT1(-/-) satellite cells proliferated slowly, but efficiently differentiated into multinuclear myotubes in vitro. Transfer of a retrovirus vector-mediated POMGnT1 gene into POMGnT1(-/-) myoblasts completely restored the glycosylation of alpha-DG, but proliferation of the cells was not improved. Our results suggest that proper glycosylation of alpha-DG is important for maintenance of the proliferative activity of satellite cells in vivo.

Pubmed ID: 19114101


  • Miyagoe-Suzuki Y
  • Masubuchi N
  • Miyamoto K
  • Wada MR
  • Yuasa S
  • Saito F
  • Matsumura K
  • Kanesaki H
  • Kudo A
  • Manya H
  • Endo T
  • Takeda S


Mechanisms of development

Publication Data

February 23, 2009

Associated Grants


Mesh Terms

  • Animals
  • Cell Proliferation
  • Cells, Cultured
  • Creatine Kinase
  • Embryonic Stem Cells
  • Fibrosis
  • Gene Deletion
  • Gene Targeting
  • Immunohistochemistry
  • Mice
  • Muscle, Skeletal
  • Muscular Dystrophy, Animal
  • Myoblasts
  • N-Acetylglucosaminyltransferases
  • Phenotype
  • Regeneration
  • Satellite Cells, Skeletal Muscle
  • Signal Transduction