Contractile properties are impaired in congenital myopathies


A hallmark of healthy muscle is the ratio between muscle strength and muscle cross-sectional area (CSA), also called the specific strength. This ratio has been used as an index of muscle quality for more than a century (1,2). It depends on muscle -architecture (pennate, multipennate, longitudinal), -fiber pennation angle, -fiber type, and -fat content (3–5). Fatty replacement of muscles is seen in many myopathies (5–8), reducing the specific strength without necessarily reducing the ability of the remaining muscle fibers to contract.


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