Minzer Karen, Lee Olivia, Hong John J, Singer Harvey S
Department of Neurology, Johns Hopkins University School of Medicine, Jefferson Street Building 1-124, 600 N. Wolfe Street, Baltimore, MD 21287, USA.
J Neurol Sci. 2004 Apr 15;219(1-2):55-61. doi: 10.1016/j.jns.2003.12.006.
The precise neuropathological mechanism underlying Tourette syndrome (TS) is unknown. In order to evaluate a variety of proposed dopaminergic abnormalities, postmortem tissue samples were obtained from three individuals with TS (two typical males with childhood onset, ages 29 and 77, and a 62-year-old female with adult-onset) and three age- and sex-matched controls. Samples from caudate, putamen, ventral striatum, and prefrontal cortex (Brodmann's area 9, BA9) were analyzed by semiquantitative immunoblotting for relative densities of dopamine receptors (D1, D2), transporter (DAT), monoamine terminals (vesicular monoamine transporter type 2), vesicular docking and release proteins (VAMP-2, synaptotagmin, SNAP-25, syntaxin, synaptophysin), and receptors inhibiting dopamine release (alpha 2-adrenergic receptors, alpha-2A). Concentrations of monoamine neurotransmitters and their metabolites were assessed by high performance liquid chromatography. Data from each TS sample was calculated as a percent value of its control. Results showed that prefrontal cortex, rather than striatum, had the greatest number of changes in the two typical TS cases, including increases for D2, DAT, VAMP-2, and alpha-2A. All three TS subjects had increased densities of prefrontal D2 receptor protein, greater than 140% of their matched control. These results suggest the presence of a prefrontal-dopaminergic abnormality in TS and emphasize the need for a more specific focus on the frontal lobe.
抽动秽语综合征(TS)潜在的精确神经病理机制尚不清楚。为了评估各种提出的多巴胺能异常情况,从三名TS患者(两名儿童期起病的典型男性,年龄分别为29岁和77岁,以及一名62岁成年期起病的女性)和三名年龄及性别匹配的对照者身上获取了死后组织样本。通过半定量免疫印迹法分析尾状核、壳核、腹侧纹状体和前额叶皮质(布罗德曼9区,BA9)样本中多巴胺受体(D1、D2)、转运体(DAT)、单胺终末(囊泡单胺转运体2型)、囊泡对接和释放蛋白(VAMP-2、突触结合蛋白、SNAP-25、 syntaxin、突触素)以及抑制多巴胺释放的受体(α2-肾上腺素能受体,α-2A)的相对密度。通过高效液相色谱法评估单胺神经递质及其代谢产物的浓度。每个TS样本的数据计算为其对照的百分比值。结果显示,在两名典型的TS病例中,前额叶皮质而非纹状体的变化最多,包括D2、DAT、VAMP-2和α-2A的增加。所有三名TS受试者前额叶D2受体蛋白密度均增加,大于其匹配对照的140%。这些结果表明TS中存在前额叶-多巴胺能异常,并强调需要更具体地关注额叶。