SERUM LEVELS OF CARBOXYTERMINAL PROPEPTIDE OF TYPE-I PROCOLLAGEN, AMINOTERMINAL PROPEPTIDE OF TYPE-III PROCOLLAGEN AND LAMININ P1 IN DUCHENNE MUSCULAR-DYSTROPHY
A. Vonmoers et al., SERUM LEVELS OF CARBOXYTERMINAL PROPEPTIDE OF TYPE-I PROCOLLAGEN, AMINOTERMINAL PROPEPTIDE OF TYPE-III PROCOLLAGEN AND LAMININ P1 IN DUCHENNE MUSCULAR-DYSTROPHY, Acta paediatrica, 86(4), 1997, pp. 377-380
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.