A perspective on the role of collagenase in recessive dystrophic epidermolysis bullosa.
The pathophysiology of tissue fragility in recessive dystrophic epidermolysis bullosa may be due in part to excessive destruction of interstitial collagens by a structurally altered, but catalytically active, form of human skin collagenase. Therapeutic attempts directed toward reducing the expression of this enzyme have resulted in clinical improvement in some patients with the disease.
Bauer
E A
EA
Department of Medicine, Washington University School of Medicine, St Louis, MO 63110.
Tabas
M
M
D004820:Epidermolysis Bullosa / Q000188:drug therapy / Q000201:enzymology* / Q000235:genetics; D005347:Fibroblasts / Q000201:enzymology; D006801:Humans; D003012:Microbial Collagenase / Q000378:metabolism*; D010672:Phenytoin / Q000627:therapeutic use
D010672:Phenytoin; D003012:Microbial Collagenase