University of California San Francisco, San Francisco, 1701 Divisadero St, San Francisco, CA 94115, USA.
Cephalalgia. 2011 Oct;31(13):1368-80. doi: 10.1177/0333102411418847. Epub 2011 Sep 5.
Familial hemiplegic migraine type 1 (FHM-1) is caused by mutations in the CACNA1A gene, with the R192Q mutation being the most common. Elevated calcitonin gene-related peptide (CGRP) levels in acute migraine and clinical trials using CGRP receptor antagonists suggest CGRP-related mechanisms are important in migraine.
Wild-type and R192Q knock-in mice were anaesthetized and perfused. Using immunohistochemical staining, the expression of CGRP in the trigeminocervical complex (TCC) and in the trigeminal and dorsal root ganglia was characterized.
There was a 38% reduction in the percentage of CGRP-immunoreactive cells in the trigeminal ganglia (p < 0.001) of R192Q knock-in mice compared to wild-type animals. The size distribution profile of CGRP-immunoreactive cells within the trigeminal ganglia demonstrated no significant difference in cell diameter between the two groups (p ≥ 0.56). CGRP expression was also reduced in thoracic ganglia of R192Q knock-in mice (21% vs. 27% in wild-type group; p < 0.05), but not in other ganglia. In addition, decreased CGRP immunoreactivity was observed in the superficial laminae of the TCC in R192Q knock-in mice, when compared to the control group (p < 0.005).
The data demonstrates that the FHM-1 CACNA1A mutation alters CGRP expression in the trigeminal ganglion and TCC. This suggests further study of these animals is warranted to characterize better the role of these mutations in the neurobiology of migraine.
家族性偏瘫性偏头痛 1 型(FHM-1)是由 CACNA1A 基因突变引起的,其中 R192Q 突变最为常见。急性偏头痛患者降钙素基因相关肽(CGRP)水平升高,以及使用 CGRP 受体拮抗剂的临床试验表明,CGRP 相关机制在偏头痛中很重要。
对野生型和 R192Q 基因敲入小鼠进行麻醉和灌注。通过免疫组织化学染色,对三叉神经根颈复合体(TCC)和三叉神经及背根神经节中的 CGRP 表达进行了特征描述。
与野生型动物相比,R192Q 基因敲入小鼠三叉神经节中 CGRP 免疫反应性细胞的百分比减少了 38%(p<0.001)。两组之间三叉神经节中 CGRP 免疫反应性细胞的大小分布曲线在细胞直径上没有显著差异(p≥0.56)。R192Q 基因敲入小鼠胸段脊神经节中的 CGRP 表达也减少(与野生型组相比为 21%对 27%;p<0.05),但其他神经节没有减少。此外,与对照组相比,R192Q 基因敲入小鼠 TCC 浅层中 CGRP 免疫反应性降低(p<0.005)。
数据表明,FHM-1 CACNA1A 突变改变了三叉神经节和 TCC 中的 CGRP 表达。这表明有必要对这些动物进行进一步研究,以更好地描述这些突变在偏头痛神经生物学中的作用。