Collagenase and stromelysin expression in recessive dystrophic epidermolysis bullosa (RDEB) was studied at both the protein and the gene expression levels in fibroblast cultures. The amount of enzyme protein in the culture medium, as determined using a specific enzyme assay, showed a 9.7-fold increa
Interleukin-1 induces collagenase production by recessive dystrophic epidermolysis bullosa fibroblasts
β Scribed by K. Nomura; T. Imaizumi; H. Mikami; K. Tamai; M. Takahashi; I. Hashimoto
- Publisher
- Springer-Verlag
- Year
- 1990
- Tongue
- English
- Weight
- 417 KB
- Volume
- 282
- Category
- Article
- ISSN
- 0340-3696
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β¦ Synopsis
We examined the direct effect of interleukin-1 (IL-1) on the collagenase production by epidermolysis bullosa (EB) fibroblasts. Addition of IL-1 at concentrations of 2.5 x 10-4 units/ml or below in the culture media greatly enhanced collagenase production by two cell lines of recessive dystrophic EB (RDEB) fibroblasts. They produced 4.82 l+ 0.04 to 5.93 _+ 0.39 units]ml of enzyme, as compared to 0.02 + 0.07 units]ml in the absence of IL-1. In contrast, collagenase production by two cell lines of dominant dystrophic EB (DDEB) and normal fibroblasts was not, or only slightly, increased up to 0.69 _+ 0.28 units]ml. IL-1 concentrations of 2.5 x 10-s units[ml or higher failed to induce collagenase production by all fibroblasts. 3Hthymidine uptake increased by about 110-376% of control after IL-1 treatment. In addition, these data were obtained using fibroblasts of the 13-15 passages, suggesting that the property might be determined genetically. Although RDEB seems to be a wide heterogeneous group, the present data strongly suggest that the property may be specific to and characteristic of some types of RDEB cells.
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Loss-of-function mutations in the gene encoding type VII collagen, COL7A1, are the molecular basis of the blistering skin disorder, recessive dystrophic epidermolysis bullosa (RDEB). COL7A1 maps to a region of the short arm of chromosome 3 that has been found to be deleted in many types of malignanc
## Communicated by Michel Goossens The Hallopeau-Siemens variant of recessive dystrophic epidermolysis bullosa (HS-RDEB) is a severe inherited skin disease characterized by the absence of collagen type VII (COLVII) and anchoring fibrils (AF), caused by mutations in collagen type VII gene (COL7A1).
Interleukin 1 and poly(r1) . poly(rC) induce hybridoma growth factor in human fibroblasts