SERUM LEVELS OF CARBOXYTERMINAL PROPEPTIDE OF TYPE-I PROCOLLAGEN, AMINOTERMINAL PROPEPTIDE OF TYPE-III PROCOLLAGEN AND LAMININ P1 IN DUCHENNE MUSCULAR-DYSTROPHY

The striking proliferation of connective tissue in Duchenne muscular d ystrophy is attributed, besides other components of the extracellular matrix, to an increase of endomysial and perimysial type III and type I collagen. We investigated if muscle fibrosis correlates to an increa sed serum concentration of procollagen I or m. Therefore, we measured the serum levels of carboxyterminal propeptide of type I procollagen, aminoterminal propeptide of type III procollagen and laminin P1 in 20 boys with progressive muscular dystrophy (16 definite Duchenne muscula r dystrophy, 2 suspected of Duchenne muscular dystrophy, 2 Becker musc ular dystrophy). In contrast to collagen I and III the expression of l aminin in the basement membrane is known to be normal in Duchenne musc ular dystrophy. There was no significant alteration of serum concentra tion of procollagen III N-peptide, procollagen I C-peptide and laminin P1 in boys with Duchenne muscular dystrophy. Measuring these paramete rs is not useful for investigating the extent of muscle fibrosis or fo r monitoring the effect of therapeutic trials such as steroid treatmen t.