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Absolute quantification of muscle glycogen content in patients with glycogen storage disease by 13C NMR spectroscopy at 7 Tesla
Author(s) -
Heinicke Katja,
Dimitrov Ivan,
Cheshkov Sergey,
Malloy Craig R.,
Haller Ronald G.
Publication year - 2012
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.26.1_supplement.1078.39
Subject(s) - glycogen , creatine , nuclear magnetic resonance spectroscopy , chemistry , in vivo magnetic resonance spectroscopy , magnetic resonance imaging , nuclear medicine , nuclear magnetic resonance , medicine , biochemistry , radiology , physics , organic chemistry
Objective Evaluate the invivo muscle glycogen content in patients with glycogen storage disease by performing noninvasive 13 C NMR spectroscopy in ultra‐high fields with the advantages of improved signal‐to‐noise ratio and spectral resolution. Methods Muscle glycogen content was measured in 3 patients with McArdle disease (MD), 1 phosphofructokinase deficient (PFKD) patient, and 4 healthy controls (C). 13 C spectra were acquired on a whole‐body 7T scanner (Philips Medical) using a partial volume human calf coil operating in quadrature for 1 H and 13 C. The coil had a small vial of acetate fixed inside, to serve as an external reference for quantification. For absolute quantification, a 1L cylindrical phantom was prepared with 100 mM glycogen, and 40 mM creatine. Results Muscle glycogen concentration was increased in MD (184.7±14.9 mM) and PFKD (196.0 mM) compared with C (73.1±13.0 mM). Creatine concentration and glycogen/creatine ratio respectively were 82.2±21.9 mM and 2.3±0.6 in MD, 120.5 mM and 1.6 in PFKD, 74.5±8.3 mM and 1.0±0.3 in C. T1 values, the strongly field dependent relaxation times, were established at 7T for glycogen 3.1±1.4 s and creatine 8.8±5.7 s in MD, 0.8±0.4 s and 2.0±1.4 s in C, respectively. Conclusions Muscle glycogen concentration was 2.5‐fold increased in patients with glycogen storage disease. Noninvasive ultra‐high filed 13 C NMR spectroscopy may provide a valuable tool to study glycogen storage in progression of the pathology and efficacy of treatment. Study supported by Muscular Dystrophy Association and Giant Tiger Foundation.