A hallmark of glycogen storage disease type II, caused by defective alpha-glucosidase (AGLU) activity, is a progressive decline in muscle performance. The objective of this study was to determine the relative contribution to this decline in muscle performance of (1) decline in muscle mass; (2) decline in muscle protein content per unit mass; and (3) accumulation of glycogen. To this end, isometric torque and power in AGLU(-/-) mice at 7, 13, and 20 months were assessed in situ. Power (approximately 24 mW) and torque (approximately 2.45 Nmm) did not change with age in control animals, but declined significantly in AGLU(-/-) mice, in the three age groups. No decline in protein content per unit muscle mass was observed. Muscle atrophy explained one third of the decline in muscle performance; the remaining part was attributed to a decrease in muscle quality--a decrease in mechanical performance per unit muscle mass. Mechanical effects of glycogen inclusions could not fully explain this decrease. Additional factors must therefore play a role.
Hesselink, R. P., van Kranenburg, G. P. J., Wagenmakers, A. J. M., van der Vusse, G. J., & Drost, M. R. (2005). Age-related decline in muscle strength and power output in acid 1-4 alpha-glucosidase knockout mice. Muscle & Nerve, 31(3), 374-381. https://doi.org/10.1002/mus.20270