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首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >Cerebellar neurons lacking complex gangliosides degenerate in the presence of depolarizing levels of potassium
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Cerebellar neurons lacking complex gangliosides degenerate in the presence of depolarizing levels of potassium

机译：缺乏复杂神经节苷脂的小脑神经元在钾去极化水平下退化

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摘要

Mice engineered to lack GM2/GD2 synthase (GalNAc-T), with resultant deficit of GM2, GD2, and all gangliotetraose gangliosides, were originally described as showing a relatively normal pheno- type with only a slight reduction in nerve conduction. However, a subsequent study showed that similar animals suffer axonal de- generation, myelination defects, and impaired motor coordination. We have examined the behavior of cerebellar granule neurons from these neonatal knockouts in culture and have found evidence of impaired capacity for Ca~2+ regulation. These cells showed relatively normal behavior when grown in the presence of phys- iological or moderately elevated K~+ but gradually degenerated in the presence of high K~+. This degeneration in depolarizing medium was accompanied by progressive elevation of intracellular calcium and onset of apoptosis, phenomena not observed with normal cells. No differences were detected in cells from normal vs. Het- erozygous mice. These findings suggest that neurons from Gal- NAc-T knockout mice are lacking a calcium regulatory mechanism that is modulated by one or more of the deleted gangliosides, and they support the hypothesis that maintenance of calcium ho- meostasis is one function of complex gangliosides during, and perhaps subsequent to, neuronal development.
机译：被设计为缺乏GM2 / GD2合酶（GalNAc-T），导致GM2，GD2和所有神经节四糖神经节苷脂缺乏的小鼠，最初被描述为表现出相对正常的表型，仅神经传导稍有减少。但是，随后的研究表明，类似的动物患有轴突变性，髓鞘缺损和运动协调障碍。我们已经检查了这些新生儿敲除物中小脑颗粒神经元的行为，并发现了Ca〜2 +调节能力受损的证据。这些细胞在生理性或适度升高的K〜+存在下生长时表现出相对正常的行为，但在高K〜+存在下逐渐退化。在去极化培养基中的这种变性伴随着细胞内钙的逐步升高和凋亡的开始，这是正常细胞未观察到的现象。在正常和纯合子小鼠的细胞中未检测到差异。这些发现表明，来自Gal-NAc-T基因敲除小鼠的神经元缺乏一种或多种缺失的神经节苷脂调节的钙调节机制，并且它们支持以下假设：维持钙质稳态是复杂神经节苷脂在体内的一种功能。 ，甚至可能在神经元发育之后。

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