Chemotactic migration of skin fibroblasts derived from different types of mucopolysaccharidoses

The mucopolysaccharidoses (MPS) are rare genetic disorders of glycosaminoglycan metabolism. Patients with theses diseases accumulate excessive amounts of several mucopolysaccharides within the lysosomes of most tissues. In an in vitro study the authors examined the chemotactic property of skin fibroblasts from different types of MPS. In a second series of experiments the authors attempted to correct the chemotactic behaviour of these cells, by preincubation with conditioned medium (CM) from human embryo fibriblasts (HEF), which contains the lacking enzymes in each specific disease. The investigation show that the storage of mucopolysaccharides in the different types of MPS is correlated with a change of another cell property, chemotactic migration. A partial restoration of chemotactic activity occurred after the addition of the lacking enzymes. Even after correction of the enzymatic defect in MPS type II, the cells do not obtain the control chemotactic capacity. This implies that in addition to increased storage of mucopolysaccharides other properties of the cells are affected.