The cytoskeleton of cultured skin fibroblasts from patients with Huntington's chorea.

Abstract

Cultured skin fibroblasts from patients with Huntington's chorea were prepared for indirect immunofluorescence using monospecific antibodies to tubulin, actin, and fibronectin. The fibroblasts were also visualized by transmission electron microscopy. The fibroblasts were observed after 3 hours of plating and treatment with various concentrations of colcemid and cytochalasin B to test the reaction of the microfibrillar network to stressful conditions. Disorders were not apparent in the cytoskeletal system (microtubules, microfilaments, and intermediate filaments) when compared with normal controls. Fibronectin was arranged in a fibrillar pattern similar to that seen with actin immunofluorescence. This colinear arrangement was not disturbed in Huntington's chorea cells. Microtubules, microfilaments, and 10-nm intermediate filaments became more parallel as the incubation period increased from 3 to 24 hours. This study showed that the cytoskeleton and the attachment of one surface protein (fibronectin) are not affected in Huntington's chorea.