Increased gene expression of matrix metalloproteinase-3 (stromelysin) in skin fibroblasts from patients with severe recessive dystrophic epidermolysis bullosa.
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.
Sawamura
D
D
Department of Dermatology, Hirosaki University, Japan.
Sugawara
T
T
Hashimoto
I
I
Bruckner-Tuderman
L
L
Fujimoto
D
D
Okada
Y
Y
Utsumi
N
N
Shikata
H
H
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
D012313:RNA; D008666:Metalloendopeptidases; D019278:Matrix Metalloproteinase 3