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在正常小鼠脊髓中鉴定出的棘神经元在 wobbler 小鼠中发生改变:一种遗传性运动神经元疾病的模型。

Spiny interneurons identified in the normal mouse spinal cord show alterations in the Wobbler mouse: a model for inherited motoneuron disease.

机构信息

Department of Anatomy, Tongji Medical University, Wuhan, 430030 (China).

出版信息

Restor Neurol Neurosci. 1992 Jan 1;4(6):381-92. doi: 10.3233/RNN-1992-4603.

DOI:10.3233/RNN-1992-4603
PMID:21551672
Abstract

Presumed interneurons are described in the Golgi-impregnated cervical spinal cord taken from normal phenotype and motoneuron-diseased mice of the Wobbler mouse strain (NFR/wr), as well as from the spinal cord of two related normal mouse strains (C57B1/6N and NFR/N). The interneurons, distributed throughout Rexed's laminae V-VIII, are characterized by numerous spines clustered along the distal dendrites. Quantitatively, the soma size (μm2) of the interneurons in the Wobbler specimens studied late in the motoneuron disease is smaller than that measured in the pair-matched (3-week-old) normal phenotype littermates. Early in the disease, the spine density (number of spines per 100 μm length dendrite) is greater compared with the normal phenotype littermates, perhaps implying that sprouting may occur. At a later stage in the disease process, the spine density does not differ significantly. However the increase in the spine density expected with advancing age is not observed for the Wobbler interneurons. It is proposed that perhaps the normal age-related proliferation of spines is impaired in the Wobbler mice. Since the measurements for spine length are lower in the Wobbler interneurons studied late in the motoneuron disease compared with the pair-matched (3-month-old) normal phenotype littermates, the normal age-related lengthening of the spines seems to be lacking. In addition, the spine length measured in the normal phenotype littermates is significantly greater compared with the normal mice (NFR/N, C57B1/6N). Thus the growth patterns of the spines may differ in the Wobbler mouse strain compared with the normal (C57B1/6N, NFR/N) mouse strains. It is proposed that the Wobbler motoneuron disease affects interneurons as well as motoneurons.

摘要

在取自正常表型和运动神经元疾病 wobbler 小鼠(NFR/wr)以及两种相关正常小鼠品系(C57B1/6N 和 NFR/N)的高尔基浸渍颈脊髓中描述了假定的中间神经元。这些中间神经元分布在 Rexed 的 laminae V-VIII 中,其特征是沿远端树突聚集着许多棘突。在研究运动神经元疾病后期的 wobbler 标本中,中间神经元的体大小(μm2)比配对匹配(3 周龄)正常表型同窝仔的体大小小。在疾病早期,与正常表型同窝仔相比,棘突密度(每 100 μm 长度树突的棘突数)更高,这可能意味着可能发生了发芽。在疾病过程的后期阶段,棘突密度没有显著差异。然而,wobbler 中间神经元没有观察到与年龄相关的棘突密度增加。据推测,也许 wobbler 小鼠中与年龄相关的棘突增殖受损。由于在研究运动神经元疾病后期的 wobbler 中间神经元中测量的棘突长度比配对匹配(3 个月龄)正常表型同窝仔低,因此正常的与年龄相关的棘突延长似乎缺乏。此外,在正常表型同窝仔中测量的棘突长度明显大于正常小鼠(NFR/N,C57B1/6N)。因此,与正常(C57B1/6N,NFR/N)小鼠品系相比,wobbler 小鼠品系中棘突的生长模式可能不同。据推测,wobbler 运动神经元疾病影响中间神经元和运动神经元。

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Spiny interneurons identified in the normal mouse spinal cord show alterations in the Wobbler mouse: a model for inherited motoneuron disease.在正常小鼠脊髓中鉴定出的棘神经元在 wobbler 小鼠中发生改变:一种遗传性运动神经元疾病的模型。
Restor Neurol Neurosci. 1992 Jan 1;4(6):381-92. doi: 10.3233/RNN-1992-4603.
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引用本文的文献

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The wobbler mouse: a neurodegeneration jigsaw puzzle.摇摆小鼠:一幅神经退行性变的拼图。
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The 21-aminosteroid U-74389F attenuates hyperexpression of GAP-43 and NADPH-diaphorase in the spinal cord of wobbler mouse, a model for amyotrophic lateral sclerosis.21-氨基类固醇U-74389F可减轻肌萎缩侧索硬化症模型摇摆小鼠脊髓中GAP-43和NADPH-黄递酶的过度表达。
Neurochem Res. 1999 Jan;24(1):1-8. doi: 10.1023/a:1020918310281.