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Age‐related decline in muscle strength and power output in acid 1–4 α‐glucosidase knockout mice
Author(s) -
Hesselink Reinout P.,
Van Kranenburg Gerrit,
Wagenmakers Anton J. M.,
Van der Vusse Ger J.,
Drost Maarten R.
Publication year - 2005
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.20270
Subject(s) - isometric exercise , glycogen , endocrinology , medicine , muscle power , muscle mass , motor unit , sarcopenia , muscle atrophy , muscle hypertrophy , glycogen storage disease type ii , biology , skeletal muscle , chemistry , anatomy , physical medicine and rehabilitation , disease , enzyme replacement therapy
Abstract A hallmark of glycogen storage disease type II, caused by defective α‐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 (∼24 mW) and torque (∼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. Muscle Nerve, 2005