Gangliosides of human spinal cord: aberrant composition of cords from patients with amyotrophic lateral sclerosis.

The ganglioside content of formalin-fixed human spinal cords and fresh human spinal cords was found to be both quantitatively and qualitatively similar. However, the spinal cord was found to be heterogeneous with respect to ganglioside content. In particular, a gradient of GD1a was observed, increasing from a low content in the cervical region to a relatively high content in the sacral region. Comparison of the ganglioside content of nine normal spinal cords and nine spinal cords from clinically diagnosed amyotrophic lateral sclerosis (ALS) patients revealed no major quantitative differences, but the unique presence of three additional gangliosides in ALS spinal cords. One of these was tentatively identified as sialosylglobotetraosylceramide and was absent from eight of nine control spinal cord samples, the sole exception being the cord from a 97-year-old female.