• type III procollagen;
  • amyotrophic lateral sclerosis;
  • immunohistochemistry;
  • skin;
  • urine

Objectives - Collagen abnormalities of skin have been reported in patients with amyotrophic lateral sclerosis (ALS). However, little is known concerning the aminoterminal propeptide of type III procollagen (PIIIP) and type III collagen in ALS. The aim of this study is to measure PIIIP, a precursor form of type III collagen, in skin and serum of ALS. Material and methods - We studied PIIIP immunoreactivity of skin and measured serum levels of PIIIP in ALS patients, and the results were compared with those of control subjects. Results - Collagen bundles in the dermis of ALS were immunohistochemically strongly positive for PIIIP as compared with those of controls. The optical density of PIIIP immunostaining reactivity in ALS patients was significantly higher than in controls, and was significantly increased with duration of illness. Serum PIIIP levels in patients with ALS were significantly increased as compared with those in diseased control subjects and those in healthy control ones, and were positively and significantly associated with duration of illness. There was an appreciable positive correlation between concentrations of serum PIIIP and the density of PIIIP immunoreactivity of skin in ALS patients. Conclusion - These data suggest that a metabolic alteration of PIIIP may take place in the skin of ALS and the increased levels of serum PIIIP may reflect the increased PIIIP immunoreactivity of skin in ALS.