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Increased mRNA expression of tissue inhibitors of metalloproteinase-1 and -2 in Duchenne muscular dystrophy.

Authors: Von Moers, A  Zwirner, A  Reinhold, A  Bruckmann, O  Van Landeghem, F  Stoltenburg-Didinger, G  Schuppan, D  Herbst, H  Schuelke, M 
Citation: von Moers A, etal., Acta Neuropathol (Berl). 2005 Mar;109(3):285-93. Epub 2004 Dec 23.
Pubmed: (View Article at PubMed) PMID:15616792
DOI: Full-text: DOI:10.1007/s00401-004-0941-0

In dystrophinopathies, disease severity is generally related to the extent of muscle fibrosis. To determine whether a decrease in matrix degradation contributes to the severe fibrosis seen in Duchenne muscular dystrophy (DMD), we quantified RNA transcript numbers for the fibrolytic matrix metalloproteinases (MMP)-1 and -2 and their natural tissue inhibitors (TIMP)-1 and -2 in DMD muscle as well as in pathological and normal controls. In addition, we investigated gelatinase (MMP-2) enzyme activity by zymography. We found an up-regulation of TIMP-1, TIMP-2 and MMP-2 RNA in DMD muscle. Zymography revealed an increase in MMP-2 activity in DMD muscle homogenates, which was absent in pathological and normal controls. Therefore, besides enhanced fibrogenesis, a disturbance of matrix degradation may play a significant role in muscle fibrosis in DMD. TIMP-1 should be investigated further as a promising target for pharmacological intervention to prevent muscle fibrosis in DMD.


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CRRD Object Information
CRRD ID: 1580161
Created: 2006-06-28
Species: All species
Last Modified: 2006-06-28
Status: ACTIVE


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