Article

The skinned single muscle fiber preparation allows investigators to directly assess the impact of neuromuscular disease on contractile function at the cellular level. It is complementary to other measurement techniques that assess muscle function on a larger scale. The single-fiber technique allows one to assess the impact of disease and/or treatment differentially on Type I, IIa, IIx, and hybrid fibers, as well as on atrophic and hypertrophic fibers. With some diseases, particular muscle fiber types are preferentially affected. When applying the technique to the study of neuromuscular disease, one must recognize that in vivo muscle strength and power generation involve multiple additional steps such as central activation of motor neurons, neural transmission, neuromuscular junction transmission, and excitation-contraction coupling, which are not addressed by the single-fiber preparation. In addition, a number of mechanical processes involved in force transduction modify single muscle fiber strength and power before it is translated into in vivo whole muscle strength and power.
Thus far, single-fiber contractile properties of patients with prior polio, ALS, myotonic and FSH dystrophies, inflammatory myopathy, and critical illness myopathy have been studied. The findings from these studies have been summarized in this article, and future research applications suggested.

Langue : ANGLAIS