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Type: Journal article
Title: Mass spectrometric quantification of glycogen to assess primary substrate accumulation in the Pompe mouse
Author: Fuller, M.
Duplock, S.
Turner, C.
Davey, P.
Brooks, D.
Hopwood, J.
Meikle, P.
Citation: Analytical Biochemistry, 2012; 421(2):759-763
Publisher: Academic Press Inc Elsevier Science
Issue Date: 2012
ISSN: 0003-2697
Statement of
Maria Fuller, Stephen Duplock, Christopher Turner, Philippa Davey, Doug A. Brooks, John J. Hopwood, Peter J. Meikle
Abstract: Glycogen storage in the α-glucosidase knockout((6neo/6neo)) mouse recapitulates the biochemical defect that occurs in the human condition; as such, this mouse serves as a model for the inherited metabolic deficiency of lysosomal acid α-glucosidase known as Pompe disease. Although this model has been widely used for the assessment of therapies, the time course of glycogen accumulation that occurs as untreated Pompe mice age has not been reported. To address this, we developed a quantitative method involving amyloglucosidase digestion of glycogen and quantification of the resulting free glucose by liquid chromatography/electrospray ionization-tandem mass spectrometry. The method was sensitive enough to measure as little as 0.1 μg of glycogen in tissue extracts with intra- and interassay coefficients of variation of less than 12%. Quantification of glycogen in tissues from Pompe mice from birth to 26 weeks of age showed that, in addition to the accumulation of glycogen in the heart and skeletal muscle, glycogen also progressively accumulated in the brain, diaphragm, and skin. Glycogen storage was also evident at birth in these tissues. This method may be particularly useful for longitudinal assessment of glycogen reduction in response to experimental therapies being trialed in this model.
Keywords: Mass spectrometry; Glycogen; Mouse; Pompe disease
Rights: © 2011 Elsevier Inc. All rights reserved.
RMID: 0020116744
DOI: 10.1016/j.ab.2011.12.026
Appears in Collections:Paediatrics publications

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