Chemotaktische Aktivität von Fibroblasten bei Mukopolysaccharidosen.

Certain clinical symptoms such as hernias or joint contracture in patients with mucopolysaccharidoses (MPS) cannot be explained as direct consequences of the disturbed glycosaminoglycan metabolism. They may be related to secondary changes of connective tissue components. The glycoprotein fibronectin is a constituent of connective tissue with a high affinity to polyanions such as heparan sulfate or heparin. Fibronectin in addition is a potent stimulus for fibroblasts to migrate chemotactically. We studied this cell property in MPS fibroblasts. The chemotactic activity of all MPS types was diminished. MPS II fibroblasts were chemotactically inactive. When the cells were corrected for the lacking enzyme by adding conditioned medium from control fibroblasts, the chemotactic migration increased except for MPS type II cells. The known enzyme defect in the degradation process of glycosaminoglycans in MPS results in lysosomal storage of degradation products and in addition causes changes of other cell properties.