Domańska-Janik K, Wikiel H, Zelman I, Strosznajder J
Neurochem Pathol. 1986 Jun;4(3):135-51. doi: 10.1007/BF02834354.
The paralytic tremor (pt) rabbit, a neurological mutant, exhibits hypomyelination transmitted in X-linked recessive fashion. This rabbit mutant was used for regional lipid analyses of different brain structures during development. There was a significant decrease of myelin-specific lipids, particularly in the cerebroside and sulfatide in pt rabbits. The decrease of phospholipid and cholesterol was relevant to the total lipids depletion. The molar ratio of galactolipid to phospholipid decreased in the pt rabbit brain in each age group examined. The other lipids typical for myelin, such as ethanolamine glycerophospholipids, sphingomyelin, and GM1 ganglioside, were also diminished in the myelin-rich structures, but were not changed in the cortical gray matter of pt rabbits. In contrast, the total amount of gangliosides was near control levels and, therefore, in the mutant rabbits, the white matter and brain stem contained a higher proportion of lipid, as ganglioside, relative to the control animals. This result suggests that neuronal membranes were not involved in this pathology. The characteristic biochemical abnormalities exhibited in the pt rabbit suggest that a defect of oligodendroglial cell function is primarily responsible for the myelin abnormality.
麻痹性震颤（pt）兔是一种神经学突变体，表现出以X连锁隐性方式遗传的髓鞘形成不足。这种兔突变体被用于发育过程中不同脑结构的区域脂质分析。髓鞘特异性脂质显著减少，尤其是pt兔脑中的脑苷脂和硫脂。磷脂和胆固醇的减少与总脂质消耗有关。在所检查的每个年龄组中，pt兔脑内半乳糖脂与磷脂的摩尔比均下降。其他典型的髓鞘脂质，如乙醇胺甘油磷脂、鞘磷脂和GM1神经节苷脂，在富含髓鞘的结构中也减少，但在pt兔的皮质灰质中没有变化。相比之下，神经节苷脂的总量接近对照水平，因此，在突变兔中，相对于对照动物，白质和脑干中神经节苷脂形式的脂质比例更高。这一结果表明神经元膜不参与这种病理过程。pt兔表现出的特征性生化异常表明，少突胶质细胞功能缺陷是髓鞘异常的主要原因。
