CHEMICAL PATHOLOGY OF GM1‐GANGLIOSIDOSIS (GENERALIZED GANGLIOSIDOSIS)
CHEMICAL PATHOLOGY OF GM1‐GANGLIOSIDOSIS (GENERALIZED GANGLIOSIDOSIS)
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GM1-神经节细胞增多症（广义神经节细胞增多症）的化学病理学
DOI：
10.1097/00005072-196901000-00003
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发表时间：
1969
影响因子：
3.2
通讯作者：
S. Kamoshita
中科院分区：
文献类型：
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作者：
Kunihiko Suzuki;Kinuko Suzuki;S. Kamoshita
Jatzkewitz 和 Sandhoff 首先提出了一种不同于 Tay-Sachs 病（G-神经节苷脂沉积症）的特定神经节苷脂沉积症的存在（30）。他们报告说，一例婴儿黑蒙性白痴患者的大脑中含有过量的正常主要单唾液酸神经节苷脂（G）。这与 Tay-Sachs 病相反，Tay-Sachs 神经节苷脂 (G) 在大脑中异常积累。 Jatzkewitz 及其同事还报告称，同一大脑中的神经酰胺四己糖苷含量异常高 (29, 30)。我们认为他们的数据只是提示性的而非确定性的，因为他们分析了在福尔马林中保存了 26 年的大脑。我们发现福尔马林在几年内几乎将所有多唾液酸神经节苷脂降解为单唾液酸神经节苷脂（86）。长期福尔马林保存后，神经酰胺四己糖苷（Gun-神经节苷脂的一种脱唾液酸衍生物）也会增加，因为单唾液酸神经节苷脂也会被福尔马林破坏，从而产生其脱唾液酸衍生物，尽管速度比多唾液酸神经节苷脂慢得多。 §
The existence of a specific gangliosidosis, different from Tay-Sachs disease (G-gangliosidosis), was first suggested by Jatzkewitz and Sandhoff (30). They reported that the brain of a case of infantile amaurotic idiocy contained an excessive amount of normal major monosialoganglioside (G). This was in contrast to Tay-Sachs disease where an abnormal accumulation of Tay-Sachs ganglioside (G) in the brain occurs. Jatzkewitz and co-workers also reported an abnormally high level of ceramide tetrahexoside in the same brain (29, 30). We consider their data as suggestive rather than definitive, because they analyzed a brain which had been stored in formalin for 26 years. We found that formalin degrades practically all multisialogangliosides to monosialogangliosides within the course of a few years (86). Increase in ceramide tetrahexoside, an asialo-derivative of Gun-ganglioside, also occurs after prolonged formalin preservation since monosialogangliosides are also destroyed by formalin to give risc to their asialo-derivatives although at a much slower rate than do the multisialogangliosides. §