Behavior of epidermolysis bullosa fibroblasts in a hydrated collagen lattice.
To determine if an altered ability to contract a hydrated collagen lattice is characteristic of fibroblasts from patients with recessive dystrophic epidermolysis bullosa (RDEB), we examined contraction by fibroblasts from normal subjects and patients with RDEB, dominant dystrophic epidermolysis bullosa (DDEB), and dominant epidermolysis bullosa simplex (DEBS). An extremely broad range of contractility (normal, poor, and hypercontraction) was observed in all types of epidermolysis bullosa (EB). When contraction in control fibroblasts was defined as the mean +/- 2 SD, (all control values were within this range) and the data were analyzed by the chi-square test, only 32% of EB cells fell within this range, with 47% poorly contractile and 21% hypercontractile. These data, derived from 34 patients, indicate that no single genetic defect resulting in altered contractility in the 3 distinct types of EB is likely. Neither cell viability, collagenase expression, nor PGE2 synthesis as correlated with gel contraction in any group. Indomethacin had no effect on contraction in RDEB. It is possible that the genetic defects in EB cause blister formation in vivo and may lead in some way to an abnormal interaction of fibroblasts with the extracellular matrix resulting in an altered collagen lattice contraction in vitro.
D002478:Cells, Cultured; D003094:Collagen; D017364:Collagenases; D015232:Dinoprostone; D004792:Enzyme Precursors / Q000378:metabolism; D004820:Epidermolysis Bullosa / Q000201:enzymology / Q000503:physiopathology*; D005347:Fibroblasts / Q000201:enzymology / Q000502:physiology; D006801:Humans; D003012:Microbial Collagenase / Q000378:metabolism; D011458:Prostaglandins E / Q000096:biosynthesis; D012016:Reference Values; D012867:Skin / Q000201:enzymology / Q000503:physiopathology*; D013997:Time Factors; D014867:Water
D004792:Enzyme Precursors; D011458:Prostaglandins E; D014867:Water; D003094:Collagen; D017364:Collagenases; C022440:procollagenase; D003012:Microbial Collagenase; D015232:Dinoprostone