Elevated ankyrin G in a plexiform neurofibroma and neuromas associated with pain
Elevated ankyrin G in a plexiform neurofibroma and neuromas associated with pain
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DOI：
10.1016/j.jocn.2003.09.023
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发表时间：
2004-11-01
影响因子：
2
通讯作者：
Kline, DG
中科院分区：
文献类型：
--
作者：
Kretschmer, T;Nguyen, DH;Kline, DG
锚蛋白G最近被证明在轴突膜的发育和再生中负责钠通道的激活。通过这种钠通道机制，锚蛋白G水平升高与自发性异位兴奋性有关，从而与神经组织的疼痛现象有关。在四种情况下检测轴突跨膜结构蛋白锚蛋白G: (a)疼痛性丛状神经纤维瘤；(b)疼痛性神经瘤；(c)无痛性神经瘤；(d)正常神经。神经纤维瘤组织来自一名18岁男性患者，他在股后皮神经上出现了剧烈疼痛的丛状神经纤维瘤，随后接受了手术。用PAGE分离样品蛋白，并用Western blot方法标记抗锚蛋白G抗体。结果。疼痛性神经纤维瘤的蛋白锚蛋白G带密度为6014，疼痛性神经瘤的蛋白锚蛋白G带密度为3557，而三种非疼痛性神经瘤的蛋白锚蛋白G带密度为3041、1988和606（平均+/- SD = 1878 +/- 1221）。7例患者8例正常神经中锚蛋白G的表达相对较少（平均SD = 411 +/- 339）。结论。我们的结果可能是疼痛性神经纤维瘤中锚蛋白G蛋白水平异常升高的第一个证据。由于锚蛋白G在可兴奋膜的发育和重塑中的多功能作用，可以假设，在神经纤维瘤和其他潜在的外周疼痛疾病中，锚蛋白G的显著增加有助于过度兴奋轴突膜的发育。@ 2004 Elsevier Ltd版权所有。
Ankyrin G has recently been shown to be responsible for activation of sodium channels in the developing and regenerating axonal membrane. Via this sodium channel mechanism, elevated ankyrin G levels have been linked with spontaneous ectopic hype rexcitability and thus with pain phenomena in nervous tissue. Ankyrin G, a transmembrane, structural protein of the axon, was examined in four conditions: (a) painful plexiform neurofibroma; (b) painful neuroma; (c) non-painful neuromas; (d) normal nerve. Neurofibroma tissue was obtained from an 18-year old male patient who developed an intensely painful, plexiform neurofibroma of the posterior femoral cutaneous nerve and subsequently underwent surgery. Sample proteins were separated by PAGE and labeled with anti-ankyrin G antibodies in a Western blot procedure. Results. The ankyrin G band density (mug) of protein for the painful neurofibroma was 6014 and was 3557 for the painful neuroma as compared to 3041, 1988 and 606 (mean +/- SD = 1878 +/- 1221) for the three non-painful neuromas. Ankyrin G expression in normal nerves (8 specimens from 7 patients) was comparatively less (mean SD = 411 +/- 339). Conclusion. Our results may represent the first evidence for abnormally increased levels of ankyrin G protein with painful neurofibromas. Due to ankyrin G's multifunctional role in the development and remodeling of excitable membranes, it can be hypothesized that the significant increase contributes to the development of hyperexcitable axonal membranes in neurofibromas and potentially other peripheral pain conditions. @ 2004 Elsevier Ltd. All rights reserved.