Byrne M C, Ledeen R W
Exp Neurol. 1983 Jul;81(1):210-25. doi: 10.1016/0014-4886(83)90169-3.
The morphopathologic abnormalities characterizing the gangliosidoses, e.g., meganeurites and aberrant secondary neurites with associated dendritic spines and synapses, show pronounced regional variation. Because gangliosides are thought to be the causative agents, we undertook to detect possible variation in concentration or composition that would correlate with those morphologic findings. A gradient in total ganglioside and GM1 concentration was found corresponding to cerebral cortex greater than caudate = thalamus greater than cerebellum, similar to the morphologic gradient. In addition, the fatty acid compositions were variable, the proportion of docosanoate (22:0) following the same gradient. The possibility that these variations in ganglioside content and composition might influence the growth of aberrant neurites and related structures is discussed.
神经节苷脂沉积症所特有的形态病理学异常，例如巨神经突以及伴有相关树突棘和突触的异常次级神经突，表现出明显的区域差异。由于神经节苷脂被认为是致病因素，我们着手检测可能与这些形态学发现相关的浓度或组成变化。发现总神经节苷脂和GM1浓度存在梯度，对应于大脑皮层大于尾状核 = 丘脑大于小脑，与形态学梯度相似。此外，脂肪酸组成是可变的，二十二烷酸（22:0）的比例遵循相同的梯度。文中讨论了神经节苷脂含量和组成的这些变化可能影响异常神经突和相关结构生长的可能性。
