Increased collagen synthesis in skin fibroblasts from patients with primary hypertrophic osteoarthropathy. Evidence for trans-activational regulation of collagen transcription.
OBJECTIVE
To investigate collagen synthesis in skin fibroblasts from patients with primary hypertrophic osteoarthropathy (HOA), a disorder characterized clinically by skin thickening.


METHODS
Collagenase-digestible protein, messenger RNA (mRNA) levels, and transcriptional activity of the alpha 1(I) procollagen gene were assessed in skin-derived fibroblast lines.


RESULTS
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.


CONCLUSIONS
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.
Padula
S J
SJ
University of Connecticut School of Medicine, Farmington.
Broketa
G
G
Sampieri
A
A
Arakawa
M
M
Matucci-Cerinic
M
M
Downie
E
E
Korn
J H
JH
D000368:Aged; D003094:Collagen / Q000096:biosynthesis* / Q000235:genetics*; D005347:Fibroblasts / Q000378:metabolism; D006801:Humans; D008297:Male; D008875:Middle Aged; D010004:Osteoarthropathy, Primary Hypertrophic / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D011347:Procollagen / Q000235:genetics; D012333:RNA, Messenger / Q000378:metabolism; D012016:Reference Values; D012867:Skin / Q000378:metabolism* / Q000473:pathology; D015533:Transcriptional Activation
D011347:Procollagen; D012333:RNA, Messenger; D003094:Collagen