Takamiya K, Yamamoto A, Furukawa K, Zhao J, Fukumoto S, Yamashiro S, Okada M, Haraguchi M, Shin M, Kishikawa M, Shiku H, Aizawa S, Furukawa K
Department of Oncology, Scientific Data Center for the Atomic Bomb Disaster, Nagasaki University School of Medicine, Nagasaki 852, Japan.
Proc Natl Acad Sci U S A. 1998 Oct 13;95(21):12147-52. doi: 10.1073/pnas.95.21.12147.
Mice, homozygous for disrupted ganglioside GM2/GD2 synthase (EC 2.4. 1.94) gene and lacking all complex gangliosides, do not display any major neurologic abnormalities. Further examination of these mutant mice, however, revealed that the males were sterile and aspermatogenic. In the seminiferous tubules of the mutant mice, a number of multinuclear giant cells and vacuolated Sertoli cells were observed. The levels of testosterone in the serum of these mice were very low, although testosterone production equaled that produced in wild-type mice. Testosterone was found to be accumulated in interstitial Leydig cells, and intratesticularly injected testosterone was poorly drained in seminiferous fluid in the mutant mice. These results suggested that complex gangliosides are essential in the transport of testosterone to the seminiferous tubules and bloodstream from Leydig cells. Our results provide insights into roles of gangliosides in vivo.
对于神经节苷脂GM2/GD2合酶（EC 2.4.1.94）基因破坏且缺乏所有复合神经节苷脂的纯合子小鼠，未表现出任何主要的神经学异常。然而，对这些突变小鼠的进一步检查发现，雄性小鼠不育且无精子生成。在突变小鼠的生精小管中，观察到许多多核巨细胞和空泡化的支持细胞。这些小鼠血清中的睾酮水平非常低，尽管睾酮生成量与野生型小鼠相同。发现睾酮在睾丸间质细胞中蓄积，并且在突变小鼠中，睾丸内注射的睾酮在生精液中的排出较差。这些结果表明，复合神经节苷脂对于睾酮从睾丸间质细胞运输到生精小管和血流至关重要。我们的结果为神经节苷脂在体内的作用提供了见解。
