title: Increased gene expression of matrix metalloproteinase-3 (stromelysin) in skin fibroblasts from patients with severe recessive dystrophic epidermolysis bullosa.
abstract: Gene expression of matrix metalloproteinase 3 (MMP-3 = stromelysin) was examined in the skin fibroblasts obtained from patients with severe recessive dystrophic epidermolysis bullosa (RDEB). Steady-state mRNA level of MMP-3 was selectively increased in the unstimulated RDEB cells by a post-transcriptional mechanism. A parallel study on the susceptibility of type VII collagen to MMPs revealed that this type of collagen is degraded by MMP-3, but not by MMP-1 (collagenase). These data suggest that MMP-3 may play an important role in the blister formation fo the skin in RDEB patients by the degradation of anchoring fibrils consisting of type VII collagen.
authors.affiliation: Department of Dermatology, Hirosaki University, Japan.
authors.forename: D
authors.initials: D
authors.lastname: Sawamura
authors.forename: T
authors.initials: T
authors.lastname: Sugawara
authors.forename: I
authors.initials: I
authors.lastname: Hashimoto
authors.forename: L
authors.initials: L
authors.lastname: Bruckner-Tuderman
authors.forename: D
authors.initials: D
authors.lastname: Fujimoto
authors.forename: Y
authors.initials: Y
authors.lastname: Okada
authors.forename: N
authors.initials: N
authors.lastname: Utsumi
authors.forename: H
authors.initials: H
authors.lastname: Shikata
mesh_terms: D015152:Blotting, Northern; D002467:Cell Nucleus / Q000378:metabolism; D002478:Cells, Cultured; D016108:Epidermolysis Bullosa Dystrophica / Q000201:enzymology / Q000235:genetics*; D005347:Fibroblasts / Q000201:enzymology; D015870:Gene Expression; D005808:Genes, Recessive; D006801:Humans; D007700:Kinetics; D019278:Matrix Metalloproteinase 3; D008666:Metalloendopeptidases / Q000235:genetics*; D009693:Nucleic Acid Hybridization; D012313:RNA / Q000235:genetics / Q000302:isolation & purification; D012867:Skin / Q000201:enzymology*; D014158:Transcription, Genetic
chemical_list: D012313:RNA; D008666:Metalloendopeptidases; D019278:Matrix Metalloproteinase 3