RGD Reference Report - Increased mRNA expression of tissue inhibitors of metalloproteinase-1 and -2 in Duchenne muscular dystrophy. - Rat Genome Database

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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.
RGD ID: 1580161
Pubmed: PMID:15616792   (View Abstract at PubMed)
DOI: DOI:10.1007/s00401-004-0941-0   (Journal Full-text)

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.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
Duchenne muscular dystrophy  IEP 1580161 RGD 
Duchenne muscular dystrophy  ISOTIMP2 (Homo sapiens)1580161; 1580161 RGD 

Objects Annotated

Genes (Rattus norvegicus)
Timp2  (TIMP metallopeptidase inhibitor 2)

Genes (Mus musculus)
Timp2  (tissue inhibitor of metalloproteinase 2)

Genes (Homo sapiens)
TIMP2  (TIMP metallopeptidase inhibitor 2)

Objects referenced in this article
Gene TIMP1 TIMP metallopeptidase inhibitor 1 Homo sapiens
Gene Timp1 tissue inhibitor of metalloproteinase 1 Mus musculus
Gene Timp1 TIMP metallopeptidase inhibitor 1 Rattus norvegicus

Additional Information