title: Elevated procollagen type I carboxyterminal propeptide production in cultured scleroderma fibroblasts.
abstract: BACKGROUND
  We recently reported that the serum concentration of procollagen type I carboxyterminal propeptide (P1CP) in patients with systemic sclereosis (SSc) was elevated. In the present study, we investigated collagen metabolism in in vitro cultured scleroderma fibroblasts by measuring P1CP levels in the culture medium.
  
  
  RESULTS
  Spontaneous P1CP production was 4.2 times higher in fibroblast cultures from patients with SSc (n = 11) than in those from healthy controls (n = 10). P1CP production in fibroblasts derived from diffuse cutaneous SSc patients was significantly greater than that from limited cutaneous SSc patients. The serum P1CP level in SSc patients was correlated with the P1CP production of cultured fibroblasts (r = 0.815, p < 0.005). Transforming growth factor beta increased P1CP production, and gamma-interferon decreased P1CP production similarly in both SSc and normal fibroblasts. In contrast, histamine dihydrochloride increased P1CP production only in SSc fibroblasts but not in controls.
  
  
  CONCLUSIONS
  These findings suggest that P1CP production in SSc fibroblasts is relevant to in vivo collagen synthesis in SSc patients.
authors.affiliation: Department of Dermatology, Faculty of Medicine, University of Tokyo, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Kikuchi
authors.forename: T
authors.initials: T
authors.lastname: Kadono
authors.forename: M
authors.initials: M
authors.lastname: Fujimoto
authors.forename: H
authors.initials: H
authors.lastname: Ihn
authors.forename: S
authors.initials: S
authors.lastname: Sato
authors.forename: K
authors.initials: K
authors.lastname: Takehara
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D002478:Cells, Cultured; D002744:Chlorpheniramine / Q000494:pharmacology; D002927:Cimetidine / Q000494:pharmacology; D003094:Collagen / Q000096:biosynthesis; D004305:Dose-Response Relationship, Drug; D005260:Female; D005347:Fibroblasts / Q000378:metabolism* / Q000473:pathology; D006632:Histamine / Q000494:pharmacology; D006801:Humans; D007371:Interferon-gamma / Q000494:pharmacology; D008297:Male; D008875:Middle Aged; D010446:Peptide Fragments / Q000032:analysis / Q000097:blood / Q000378:metabolism*; D011347:Procollagen / Q000032:analysis / Q000097:blood / Q000378:metabolism*; D012594:Scleroderma, Localized / Q000378:metabolism* / Q000473:pathology; D012595:Scleroderma, Systemic / Q000378:metabolism* / Q000473:pathology; D016212:Transforming Growth Factor beta / Q000494:pharmacology
chemical_list: D010446:Peptide Fragments; D011347:Procollagen; D016212:Transforming Growth Factor beta; C042813:procollagen type I carboxy terminal peptide; D002744:Chlorpheniramine; D002927:Cimetidine; D006632:Histamine; D007371:Interferon-gamma; D003094:Collagen