Abstract
To investigate collagen synthesis in skin fibroblasts from patients with primary hypertrophic osteoarthropathy (HOA), a disorder characterized clinically by skin thickening. Collagenase-digestible protein, messenger RNA (mRNA) levels, and transcriptional activity of the alpha 1(I) procollagen gene were assessed in skin-derived fibroblast lines. Compared with fibroblasts from uninvolved skin, fibroblasts from involved skin had elevated levels of collagen synthesis and alpha 1(I) procollagen mRNA, and increased transcriptional activity of the alpha 1(I) procollagen promoter. Abnormalities of collagen synthesis in fibroblasts from patients with primary HOA can be accounted for, at least in part, by a trans-activated up-regulation of collagen transcription.
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