PROTEOLYTIC enzyme expression was studied by matrix metalloproteinases (MMP
) immunoreactivity (-IR) in muscle biopsies from patients with amyotrophic
lateral sclerosis (ALS), spinal muscle atrophy (SMA) and chronic axonal neu
ropathies (CANP). In normal muscle MMP-2-, MMP-7-, and MMP-9-IR was localiz
ed at neuromuscular junctions, in vessels and nerve branches. ALS biopsies
of clinically non-affected muscles revealed neither MMP-2, -7-IR nor MMP-9-
IR in atrophied myofibers. ALS-affected biopsies revealed MMP-9-IR, and to
lesser extent MMP-2- and MMP-7-IR at normal sized and atrophied myofibers.
Biopsies of SMA showed MMP-9- and MMP-7-IR at normal sized and atrophic myo
fibers, while MMP-2-IR was undetectable. In CANP MMP-9-IR was found at norm
al sized and atrophied myofibers. Distinct expression patterns of MMPs may
thus reflect different stages of muscle denervation atrophy. (C) 1999 Lippi
ncott Williams & Wilkins.