Reversible rearrangement of vimentin-type intermediate filaments in cultured human skin fibroblasts from patients with lysosomal storage diseases.

Immunofluorescence microscopy shows that unlike cytoplasmic microtubules (MT), vimentin-type intermediate filaments (IF) are collected into ring-shaped structures in affected fibroblasts. The altered IF organization could be observed in monolayers of polarized fibroblasts in the prolonged stationary growth phase and-after replating-upon their initial spreading. Transition from a discoid to an extended cellular form is accompanied by centrifugal dislocation of ring-shaped IF structures towards the cell's active edge with gradual restoration of the radial fibrillar vimentin network. Spreading of affected cells occurred more slowly than that of control fibroblasts.