Oral involvement of recessive dystrophic epidermolysis bullosa inversa
โ Scribed by Wright, J. Timothy ;Fine, Jo-David ;Johnson, Loraine B. ;Steinmetz, Todd T.
- Publisher
- John Wiley and Sons
- Year
- 1993
- Tongue
- English
- Weight
- 572 KB
- Volume
- 47
- Category
- Article
- ISSN
- 0148-7299
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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
Absent or defective collagen VII at the dermo-epidermal junction is the hallmark of dystrophic recessive epidermolysis bullosa. Little is known of the alterations of other collagenous and non-collagenous components of the basement membrane; it is likely that their assembly may be disturbed by the la
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