Skeletal Muscle Remodelling as a Function of Disease Progression in Amyotrophic Lateral Sclerosis

Figure 3

Skeletal muscle of ALS patients presents with atrophy of both type I and type II fibres. (a and b) Representative images of immunohistochemical stainings for fibre type distribution show MHC-slow in blue, MHC-fast in red, and mixed/changing fibres in purple. Atrophic fibres of both type I and type II can be seen in the same individual (b, arrow). (c) Control samples show even distribution of fibres with similar size and no mixed fibres. (e–h) Representative images of fibre type grouping and fibre type-specific atrophy in ALS muscle. (I and j) Frequency distribution of cross-sectional area at BL and 12 wk. for type I (i) and type II (j) fibres. CSA: cross-sectional area. Significant differences between BL and 12 wk. in ALS in CSA for both fibre types are indicated by . for ALS and for controls. Scale bar = 50 μm.