Growth of ectopic dendrites on cortical pyramidal neurons in neuronal storage diseases correlates with abnormal accumulation of GM2 ganglioside.
Ganglioside analysis and quantitative Golgi studies of the cerebral cortex of cats with ganglioside and nonganglioside lysosomal storage diseases reveal a correlation between the amount of accumulated GM2 ganglioside and the extent of ectopic dendrite growth on cortical pyramidal neurons. This correlation was not observed with any of the other gangliosides assayed for, including GM1 ganglioside. These results suggest a specific role for GM2 ganglioside in the initiation of ectopic neurites on pyramidal cells in vivo and are consistent with the developing hypothesis that different gangliosides have specific roles in different cell types dependent upon the receptor or other effector molecules with which they may interact.
Siegel
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DA
Department of Neuroscience, Rose F. Kennedy Center for Research in Mental Retardation and Human Development, Albert Einstein College of Medicine, Bronx, New York 10461.
Walkley
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SU
D000375:Aging / Q000378:metabolism; D000818:Animals; D002239:Carbohydrate Metabolism, Inborn Errors / Q000378:metabolism / Q000473:pathology / Q000662:veterinary; D002371:Cat Diseases; D002415:Cats; D002540:Cerebral Cortex / Q000254:growth & development / Q000378:metabolism* / Q000473:pathology*; D003712:Dendrites / Q000473:pathology / Q000502:physiology*; D005678:G(M2) Ganglioside / Q000378:metabolism*; D005733:Gangliosidoses / Q000378:metabolism / Q000473:pathology / Q000662:veterinary; D017966:Pyramidal Cells / Q000378:metabolism* / Q000473:pathology; D012016:Reference Values
D005678:G(M2) Ganglioside