Pattern of collagen synthesis and chemotactic response of fibroblasts derived from mucopolysaccharidosis patients.
For comparative studies on the migratory potential we screened fibroblast strains derived from mucopolysaccharidosis (MPS) patients regarding their differential response to chemotactic stimuli and analysed their production of extracellular matrix components. Indirect immunofluorescence staining of MPS-fibroblasts showed the same distribution of type I and type III collagen and of fibronectin as in controls. Biochemical quantification of type I and type III collagen demonstrated an unaltered ratio of these collagen types, although the total amount of newly synthesized collagens was slightly reduced in fibroblasts from MPS patients. Whereas the synthesis of major extracellular matrix components was close to normal, the response of the MPS cells to chemotactic stimuli was greatly affected. Chemotactic migration was improved when fibroblasts were pretreated with medium conditioned by normal fibroblasts, although they never reached normal levels.
D002478:Cells, Cultured; D002633:Chemotaxis; D002648:Child; D003094:Collagen / Q000096:biosynthesis*; D005260:Female; D005333:Fetus; D005347:Fibroblasts / Q000502:physiology; D005455:Fluorescent Antibody Technique; D006801:Humans; D007700:Kinetics; D009083:Mucopolysaccharidoses / Q000473:pathology / Q000503:physiopathology*; D011247:Pregnancy; D011347:Procollagen / Q000096:biosynthesis; D012867:Skin / Q000166:cytology / Q000378:metabolism
D011347:Procollagen; D003094:Collagen