Effect of purkinje cell loss on cerebellar gangliosides in nervous mutant mice

✍ Scribed by Dr. T. N. Seyfried; D. J. Bernard; R. K. Yu

• Publisher: John Wiley and Sons
• Year: 1987
• Tongue: English
• Weight: 483 KB
• Volume: 17
• Category: Article
• ISSN: 0360-4012
• DOI: 10.1002/jnr.490170308

✦ Synopsis

The distribution of cerebellar gangliosides was studied in adult (73 +/- 2 days) nervous (nr/nr) mutant mice which lose 50-90% of their Purkinje cells. This neuronal loss is associated with significant reductions in cerebellar weight and ganglioside concentration. The cerebellar dry weights (mg) and the ganglioside concentrations (microgram N-acetylneuraminic acid per 100 mg dry weight) in nr/nr mice and age-matched normal littermates (+/?) are 7.4 +/- 0.3 mg and 13.2 +/- 0.4 mg; and 411.7 +/- 4.8 micrograms and 438.5 +/- 2.1 micrograms, respectively. Abnormalities were also observed for the concentration of certain ganglioside species. Most notably, GT1a is significantly reduced by 42%, and GD3 is significantly increased by 29% in the nr/nr mice compared to the +/? mice. The nr/nr mice also express a slight but significant reduction in GT1b. No ganglioside abnormalities were observed between the nr/nr and +/? mice in cerebral cortex. We previously found reduced cerebellar GT1a content in other mutants that also lose Purkinje cells, i.e., sg/sg, pcd/pcd, and Lc/+. GT1a is not reduced, however, in wv/wv mice that lose mostly granule cells. The findings in nr/nr mice are therefore consistent with our hypothesis that GT1a is enriched in Purkinje cells. GD1a, which is enriched in mature granule cells, is not reduced in the nr/nr mice.(ABSTRACT TRUNCATED AT 250 WORDS)