Detection of glycogen in a glycogen storage disease by 13C nuclear magnetic resonance

AbstractThe livers of gsd/gsd rats homozygous for the glycogen storage disease phosphorylase b kinase deficiency were observed by 13C NMR using a surface coil. Clear signals were detected from glycogen. The concentration of glycogen as determined by NMR was ∼3-times that found in normal strains agreeing well with chemical determinations Starvation did not significantly reduce the glycogen content of the livers with glycogen storage disease whereas it reduced the signal below detectability in normal rats. Difference spectra of starved normal rats from fed gsd/gsd rats gave spectra similar in appearance to that of purified glycogen. Glycogen both in vivo and in vitro is fully visible using 13C NMR