Collagen biosynthesis in a case of infantile myofibromatosis

Abstract

Cells from a skin nodule from a patient with a recurrent form of familial myofibromatosis were cultivated in vitro. A metabolic study showed that these cells behaved like fibroblasts with collagen synthesis, a normal percentage of type III collagen, hydroxylation rate and the ability to contract a collagen gel. The main disturbances were the decreased synthesis and increased cell multiplication rate after a lag phase. This behavior was compared with that of a fibroblast culture from normal skin.