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囊性纤维化跨膜传导调节因子mRNA及其蛋白在大鼠脑中的表达与定位

Expression and localization of the cystic fibrosis transmembrane conductance regulator mRNA and its protein in rat brain.

作者信息

Mulberg A E, Resta L P, Wiedner E B, Altschuler S M, Jefferson D M, Broussard D L

机构信息

Division of Gastroenterology and Nutrition, Children's Hospital of Philadelphia, Pennsylvania 19104, USA.

出版信息

J Clin Invest. 1995 Jul;96(1):646-52. doi: 10.1172/JCI118080.

Abstract

In previous studies we have characterized the expression of the cystic fibrosis transmembrane conductance regulator (CFTR) protein in clathrin-coated vesicles derived from bovine brain and in neurons of rat brain. In this study we have further characterized the expression of the CFTR protein mRNA and protein in rat brain with reverse transcriptase polymerase chain reaction amplification (RT-PCR), in situ hybridization, and immunocytochemistry. The expression of CFTR mRNA and protein in discrete areas of brain, including the hypothalamus, thalamus, and amygdaloid nuclei, which are involved in regulation of appetite and resting energy expenditure, is identical. The presence of CFTR in neurons localized to these regions of brain controlling homeostasis and energy expenditure may elucidate the pathogenesis of other nonpulmonary and gastrointestinal manifestations which commonly are observed in children with cystic fibrosis. Dysregulation of normal neuropeptide vesicle trafficking by mutant CFTR in brain may serve as a pathogenic mechanism for disruption of homeostasis.

摘要

在先前的研究中,我们已经对源自牛脑的网格蛋白包被小泡以及大鼠脑神经元中囊性纤维化跨膜传导调节因子(CFTR)蛋白的表达进行了表征。在本研究中,我们通过逆转录聚合酶链反应扩增(RT-PCR)、原位杂交和免疫细胞化学进一步表征了大鼠脑中CFTR蛋白mRNA和蛋白的表达。CFTR mRNA和蛋白在脑的离散区域,包括参与食欲和静息能量消耗调节的下丘脑、丘脑和杏仁核中的表达是相同的。CFTR存在于定位于这些控制体内平衡和能量消耗的脑区域的神经元中,这可能阐明了囊性纤维化患儿中常见的其他非肺部和胃肠道表现的发病机制。脑中突变的CFTR对正常神经肽小泡运输的调节异常可能是体内平衡破坏的致病机制。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4176/185240/408a0711e874/jcinvest00013-0665-a.jpg

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