A. Matsumoto et al., GANGLIOSIDE CHARACTERIZATION OF A CELL-LINE DISPLAYING MOTOR NEURON-LIKE PHENOTYPE - GM2 AS A POSSIBLE MAJOR GANGLIOSIDE IN MOTOR-NEURONS, Journal of the neurological sciences, 131(2), 1995, pp. 111-118
We have examined ganglioside compositions and the presence of sulfated
glucuronyl glycolipids of immortalized motor neuron-like cell lines,
neuroblastoma-spinal cord (NSC) hybrid cell lines established by fusin
g mouse neuroblastoma N18TG2 with motor neuron-enriched embryonic spin
al cord cells. Among NSC cell lines, only NSC-34 aggregates acetylchol
ine receptors on co-cultured myotube and expresses a receptor for S-la
minin, a neuromuscular junction specific basal lamina protein. GM2, wh
ich is only a minor ganglioside component of CNS, was the major compon
ent in NSC-34 occupying almost 75% of total gangliosides, whereas GD1a
and GM3 were major species in the parental N18TG2, which had only 8.5
% GM2. These results indicated that NSC lines have unique ganglioside
pattern that is distinctive from other nervous tissues, and this patte
rn, especially that of NSC-34 cells, might reflect the characteristics
of mouse spinal motor neuron gangliosides. Sulfated glucuronyl paragl
oboside was demonstrated to be present in N18TG2, however, it could no
t be detected in either of NSC cell lines. Even though the pathogenesi
s of amyotrophic lateral sclerosis remains unknown, autoimmunological
participation has been suggested. Because high-titered antibody agains
t GM2 has been observed in a patient with amyotrophic lateral sclerosi
s-like disease, GM2 which is possibly expressed on the surface of moto
r neurons might serve as a potential target antigen in this disorder.