Kundu S K, Harati Y, Misra L K
Biochem Biophys Res Commun. 1984 Jan 13;118(1):82-9. doi: 10.1016/0006-291x(84)91070-2.
Gangliosides of healthy and pathologic muscles (amyotropic lateral sclerosis and facio-scapulo-humeral muscular dystrophy) were studied. Total ganglioside content of the affected muscles was approximately 2 fold higher than the unaffected muscles. Our results showed that ALS muscle contained a ganglioside which was absent in the unaffected and FSH muscular dystrophic muscles. Based on the results of hydrolysis with Vibrio cholerae neuraminidase and subsequent reactivity of the asialo derivative towards anti-globotetraosylceramide, we propose that the ALS ganglioside is sialosylglobtetraosylceramide, NeuAc(alpha 2-3)Ga1NAc(beta 1-3)Ga1(alpha 1-4)Ga1(beta 1-4)G1c-Cer.
对健康肌肉和患病肌肉（肌萎缩侧索硬化症和面肩肱型肌营养不良症）的神经节苷脂进行了研究。患病肌肉的总神经节苷脂含量比未患病肌肉大约高2倍。我们的结果表明，肌萎缩侧索硬化症肌肉中含有一种在未患病肌肉和面肩肱型肌营养不良症肌肉中不存在的神经节苷脂。基于霍乱弧菌神经氨酸酶水解结果以及去唾液酸衍生物对抗球四糖神经酰胺的后续反应性，我们提出肌萎缩侧索硬化症神经节苷脂是唾液酸基球四糖神经酰胺，NeuAc(α2-3)GalNAc(β1-3)Gal(α1-4)Gal(β1-4)Glc-Cer。
