Cystic fibrosis transmembrane conductance regulator expression in human spinal and sympathetic ganglia

Cystic fibrosis transmembrane conductance regulator expression in human spinal and sympathetic ganglia
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DOI:
10.1038/labinvest.2009.28
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发表时间:
2009-06-01
影响因子:
5
通讯作者:
Gu, Jiang
Gu, Jiang
中科院分区:
医学2区
文献类型:
--
作者:
Niu, Na;Zhang, Jie;Gu, Jiang

文献摘要

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囊性纤维化跨膜传导调节因子(CFTR)是一种氯离子通道蛋白,其基因突变引起囊性纤维化。已知CFTR在呼吸道、消化道和生殖道的上皮细胞中表达。它也存在于大鼠神经元和心脏神经节细胞中。在人类中,它在下丘脑中表达,但尚未在人类神经系统的其他部分中发现。在这项研究中,免疫组织化学,双染色免疫荧光,原位杂交,巢式逆转录-PCR和相对定量的实时PCR分析进行脊髓和交感神经节从7人尸检没有已知的神经系统疾病。CFTR蛋白在大部分神经节细胞中均有表达,但不同部位神经节中CFTR mRNA转录量无明显差异。我们的结论是,CFTR蛋白及其mRNA广泛表达在人类脊髓和交感神经节细胞在相对恒定的水平,可能是重要的生理和病理条件。此外,神经节中的CFTR可能与囊性纤维化中观察到的病理生理变化相关。实验室调查(2009)89,636-644; doi:10.1038/labinvest.2009.28;在线发表2009年3月30日
Cystic fibrosis transmembrane conductance regulator ( CFTR) is a chloride channel protein, and mutations of its gene cause cystic fibrosis. CFTR is known to be expressed in epithelial cells of the respiratory, digestive and reproductive tracts. It is also present in rat neurons and heart ganglion cells. In humans, it is expressed in the hypothalamus, but has not been identified in other parts of the human nervous system. In this study, immunohistochemistry, double-staining immunofluorescence, in situ hybridization, nested reverse transcription-PCR and relative quantification of real-time PCR analysis were performed on spinal and sympathetic ganglia from seven human autopsies with no known nervous system disease. CFTR protein was expressed in most ganglion cells with no obvious difference in the amounts of mRNA transcript in ganglia of different sites. We conclude that CFTR protein and its mRNA were extensively expressed at relatively constant levels in human spinal and sympathetic ganglion cells, and may be important in physiological and pathological conditions. Moreover, CFTR in ganglia may be associated with pathophysiological changes seen in cystic fibrosis. Laboratory Investigation (2009) 89, 636-644; doi:10.1038/labinvest.2009.28; published online 30 March 2009