Altered collagen and glycosaminoglycan syntheses in familial scleroderma skin fibroblasts.
BACKGROUND
The main clinical symptoms of systemic scleroderma are caused by an accumulation of collagen in the tissues. Familial scleroderma is a very rare disorder in which systemic scleroderma occurs in the members of the same family.


OBJECTIVE
To know whether or not the biosynthetic properties of familial scleroderma skin fibroblasts are similar to those of nonfamilial scleroderma fibroblasts, syntheses of extracellular matrix components in the two fibroblast strains obtained from familial scleroderma patients were studied.


METHODS
Expression of collagen and glycosaminoglycans by cultured familial scleroderma fibroblasts was determined.


RESULTS
Collagen synthesis by patient fibroblasts was elevated 1.4-fold compared with control fibroblasts. Translational activity of type I procollagen mRNA and the level of alpha 1 (I) mRNA in the patient fibroblasts was increased 1.7- and 1.6-fold, respectively. The ratio of hyaluronic acid/dermatan sulfate in the patient fibroblasts was lower than that in control fibroblasts.


CONCLUSIONS
The results indicate that the biosynthetic phenotypes of familial scleroderma fibroblasts are similar to those of nonfamilial scleroderma fibroblasts which have already been reported.
Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
S
S
Tajima
H
H
Yamada
D000328:Adult; D000368:Aged; D003094:Collagen / Q000096:biosynthesis* / Q000235:genetics; D003871:Dermatan Sulfate / Q000096:biosynthesis; D005192:Family Health; D005260:Female; D005347:Fibroblasts / Q000378:metabolism; D006025:Glycosaminoglycans / Q000096:biosynthesis*; D006801:Humans; D006820:Hyaluronic Acid / Q000096:biosynthesis; D008875:Middle Aged; D010641:Phenotype; D012333:RNA, Messenger / Q000378:metabolism; D012595:Scleroderma, Systemic / Q000235:genetics / Q000378:metabolism*; D012867:Skin / Q000378:metabolism* / Q000473:pathology
D006025:Glycosaminoglycans; D012333:RNA, Messenger; D003871:Dermatan Sulfate; D006820:Hyaluronic Acid; D003094:Collagen