Neuropediatrics 2005; 36 - P106
DOI: 10.1055/s-2005-868091

Expression of procollagen I, III und IV and their regulators in muscles of children with Cerebral Palsy (CP)

I Borggraefe 1, J Böhmer 1, S Wullinger 2, S Schroeder 1, P Bernius 3, F Heinen 1
  • 1Dr. von Haunersches Kinderspital, Ludwig-Maximilian-Universität, Pädiatrische Neurologie und Entwicklungsneurologie, München
  • 2Dr. von Haunersches Kinderspital, Ludwig-Maximilian-Universität, Molekularbiologie und Biochemische Genetik, München
  • 3Orthopädische Klinik Harlaching, Zentrum Kinderorthopädie, München

Objective: Patients with Cerebral Palsy often develop severe muscle contractures during the first two decades of life. The aim of the study was to examine mRNA-expression of extracellular matrix molecules and their regulators as their accumulation may contribute to the loss of dynamic features of the spastic muscle.

Methods: Patients with CP (n=15, Average age 10, defined grade of spasticity) who underwent surgery for severe muscle contractures were chosen for muscle specimen collection. RNA from the ham string muscles was isolated and transcribed into cDNA. Primer of molecules known to be involved in other types of tissue fibrosis were designed to detect fragments including more than one exon. The assessment of quantitative mRNA-expression was performed by the approach of Real-Time PCR.

Results: There was a significant elevated expression of mRNA of the extracellular matrix molecules procollagene I and III. Procollagen IV, which is associated with the basal lamina, also revealed a slight elevated expression. Decorin, involved in further extracellular processing of collagen, was also higher expressed when compared to the control group. Furthermore there were elevated levels of mRNA of the fibroblast activators FGF-2 and TGF-β. Some signal transduction molecules and transcription factors involved in activation of the procollagene I promotor were not differentially expressed when compared to the control group (Smad-2, -4, AP-1).

Conclusions: (1) The accumulation of collagen in the muscle of patients with CP could be due to an elevated expression of collagen I and III mRNA. (2) TGF-ß and FGF-2 may play a role in activation of collagen synthesis in this setting. (3) Although some signal transduction molecules and transcription factors involved in the activation of the procollagen I promotor were not differentially expressed it does not rule out a functional role in collagen gene expression activation in the muscle of children with CP as the functional state of these molecules is not determined by mRNA-levels alone.