HEPARAN SULFATE-LIKE IMMUNOREACTIVITY IN THE SPINAL-CORD IN MOTOR-NEURON DISEASE

The spinal cords from eight autopsy cases of sporadic motor neuron dis ease (MND) and two control cases were immunohistochemically examined u sing antibodies directed to neurofilament proteins (anti-Nf) and to he paran sulfate (HepSS-1). Variable numbers of spheroids were observed i n the anterior horns in the MND cases. In one case of MND, one third t o half of the remaining anterior horn cells contained conglomerate inc lusions in their perikarya. These pathological structures were not enc ountered in the control cases. The immunohistochemical study revealed that both anti-Nf and HepSS-1 intensely labelled all spheroids and con glomerate inclusions in the MND cases. The colocalization of heparan s ulfate with neurofilamentous accumulation suggests that heparan sulfat e is required for the aggregation of neurofilaments, resulting in the formation of spheroids and conglomerate inclusions in MND.