Collagen Metabolism in Cutis Laxa Fibroblasts: Increased Collagenase Gene Expression Associated with Unaltered Expression of Type I and Type III Collagen

Hatamochi, Atsushi; Wada, Tamiko; Takeda, Koji; Ueki, Hiroaki; Kawano, Shoji; Terada, Kihei; Morita, Tetsuro

doi:10.1111/1523-1747.ep12481519

Cited by 12 publications

(9 citation statements)

References 30 publications

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“…Electron-microscopic examination of cutis laxa has shown a decrease in elastin expression, a structural component of elastic fibers, making microfibrils visible [4]. Biochemical studies have also reported that elastin mRNA levels are reduced in several fibroblast strains of cutis laxa [7, 8, 9]. …”

Section: Discussionmentioning

confidence: 99%

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Costello Syndrome with Decreased Gene Expression of Elastin in Cultured Dermal Fibroblasts

et al. 2000

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We report a case of Costello syndrome. A 2-year-old Japanese boy presented with a ‘coarse‘ face, curly hair and loose skin of the dorsal aspect of the hands and feet with dark pigmentation. A skin biopsy of the dorsal aspect of the left hand revealed hyperkeratosis and papillomatosis of the epidermis, hyperpigmentation of the basal layer, and shortening and rupture of elastic fibers of the dermis. Electron microscopy of dermal elastic fibers showed a decreased amount of elastin with an exposed appearance of microfibrils. In Northern blot analysis of cultured dermal fibroblasts, elastin mRNA levels were reduced, suggesting a decrease in elastin production at the lesions of loose skin.

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Section: Discussionmentioning

confidence: 99%

“…In cutis laxa, morphological studies showed a reduction of elastic structures and fragmentation of elastic fibers [4, 5, 6]. Elastin mRNA levels are also reduced in several fibroblast strains of cutis laxa [7, 8, 9]. Very few studies on dermal elastic fibers in Costello syndrome have been published so far.…”

Section: Introductionmentioning

confidence: 99%

Costello Syndrome with Decreased Gene Expression of Elastin in Cultured Dermal Fibroblasts

et al. 2000

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“…Three days after the cultivation, the media and cells were harvested and stocked at -80°C until use. The MMP-1 activity was assayed using FITC-labeled type I collagen fibrils as described previously [34,35]. The activation of latent collagenase was performed by adding trypsin (4 Ìg/ml at the final concentration), the mixture was kept standing for 10 min at 37°C and then soybean trypsin inhibitor (400 Ìg/ml) was added to inhibit the trypsin activity.…”

Section: Northern Blot Analysis and Activity Assay For Mmp-1mentioning

confidence: 99%

The Suppressive Effect of Tecogalan Sodium on in vitro Angiogenesis via the Periendothelial Proteolytic Activities

et al. 2000

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The exact mechanisms of the angiostatic effect by tecogalan sodium (TS) remain unclear. We examined the effects of TS on in vitro angiogenic activity, proteolytic activity and proliferation of retinal vascular endothelial cells (RECs). TS markedly inhibited the in vitro angiogenic activity of RECs although the growth inhibition of RECs was small. TS apparently decreased the cell-associated urokinase-type plasminogen activator (uPA) activity and matrix metalloprotease 1 (MMP-1) activity even in the presence of anti-bFGF IgG. Thus, the suppression of the periendothelial matrix-degrading activities related to uPA and MMP-1 is suggested to be another possible mechanism of the antiangiogenic effect of TS, besides its prevention of bFGF REC binding which has previously been reported.

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“…C1 and C2 were obtained from the abdominal skin of two 2-year-old girls with a diagnosis of CL. 12,15 The other strain (C3) was obtained from the Human Genetic Mutant Cell Repository (Camden, NJ, U.S.A.) (GM02768, from the skin of a 4-month-old girl). The clinical diagnosis was confirmed by a skin biopsy that revealed a paucity of dermal elastic fibres.…”

Section: Fibroblast Culturesmentioning

confidence: 99%

“…8 With regard to collagen fibres, occasional irregularities in collagen morphology have been detected in CL skin, 3 and small and separated collagen bundles and fibrils of varied diameters 5,9,10 and aggregated fibrils have been reported in CL skin. Although abnormalities in collagen synthesis and the mRNA levels of type I and type III collagen have not been detected, 11,12 increased mRNA levels of collagenase, a matrix metalloproteinase (MMP) with the unique ability to initiate collagen degradation, associated with increased collagenase activity, have been found in fibroblasts derived from the skin of CL patients. 12 It has been suggested that overexpression of collagenase (MMP-1) in CL fibroblasts may contribute to the finding of abnormal collagen fibres in this disease.…”

mentioning

confidence: 99%

Regulation of matrix metalloproteinase (MMP) expression in cutis laxa fibroblasts: upregulation of MMP‐1, MMP‐3 and MMP‐9 genes but not of the MMP‐2 gene

Hatamochi

Kuroda

Shinkai

et al. 1998

British Journal of Dermatology

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A major histopathological abnormality in cutis laxa (CL) is a paucity of elastic structures. The aim of this study was to investigate the gene expression levels of the major matrix degrading factors matrix metalloproteinase (MMP) 1, MMP-2, MMP-3 and MMP-9 in CL. The gene expression levels of MMP-1, MMP-2, MMP-3 and MMP-9 in cultured CL fibroblasts were measured by northern blot, immunoblot and gelatin zymographic analysis. Markedly increased mRNA levels of MMP-1 (8.4-fold), MMP-3 (7.2-fold) and MMP-9 (more than 10-fold) were found in CL fibroblasts, whereas MMP-2 mRNA levels in these fibroblasts were unaltered. Increased protein production levels of MMP-1 (4.6-fold) and MMP-3 (5.1-fold) in CL fibroblasts were shown by immunoblot analysis. On gelatin zymographic analysis, the gelatinolytic activities of MMP-9 but not of MMP-2 were increased (2.2-fold). These results suggest that increased gene expression levels of MMP-1, MMP-3 and MMP-9 in CL fibroblasts may contribute to the histopathological abnormality in CL.

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Collagen Metabolism in Cutis Laxa Fibroblasts: Increased Collagenase Gene Expression Associated with Unaltered Expression of Type I and Type III Collagen

Cited by 12 publications

References 30 publications

Costello Syndrome with Decreased Gene Expression of Elastin in Cultured Dermal Fibroblasts

Costello Syndrome with Decreased Gene Expression of Elastin in Cultured Dermal Fibroblasts

The Suppressive Effect of Tecogalan Sodium on in vitro Angiogenesis via the Periendothelial Proteolytic Activities

Regulation of matrix metalloproteinase (MMP) expression in cutis laxa fibroblasts: upregulation of MMP‐1, MMP‐3 and MMP‐9 genes but not of the MMP‐2 gene

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