Cystic fibrosis transmembrane conductance regulator protein expression in brain.

Cystic fibrosis transmembrane conductance regulator protein expression in brain.
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DOI:
10.1097/00001756-199408150-00035
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发表时间:
1994-08
期刊:
影响因子:
1.7
通讯作者:
A. Mulberg;E. Wiedner;X. Bao;J. Marshall;D. Jefferson;S. Altschuler
A. Mulberg;E. Wiedner;X. Bao;J. Marshall;D. Jefferson;S. Altschuler
中科院分区:
医学4区
文献类型:
--
作者:
A. Mulberg;E. Wiedner;X. Bao;J. Marshall;D. Jefferson;S. Altschuler

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囊性纤维化跨膜传导调节蛋白(CFTR)已被确定在牛脑网格蛋白包被的囊泡,大鼠脑和人神经母细胞瘤细胞系使用亲和纯化的多克隆肽抗体对CFTR。间脑、中脑、脑桥和延髓的神经元的多个树突和索马体的免疫细胞化学染色也已被证实。来自大鼠脑、神经母细胞瘤细胞和牛脑网格蛋白包被囊泡的全细胞裂解物和膜表达成熟的150-165 kDa和130 kDa非糖基化形式的CFTR。CFTR定位于控制体内平衡和能量消耗的脑区域可能与囊性纤维化的非肺部表现的发病机制有关。CFTR在神经元和包被囊泡中的表达表明突变CFTR可能对神经肽囊泡运输产生影响。
The cystic fibrosis transmembrane conductance regulator protein (CFTR) has been identified in bovine brain clathrin-coated vesicles, rat brain and a human neuroblastoma cell line using affinity-purified polyclonal peptide antibodies against CFTR. Immunocytochemical staining of multiple dendrites and soma of neurons of the diencephalon, midbrain, pons and medulla oblongata, has also been demonstrated. Whole cell lysates and membranes derived from rat brain, neuroblastoma cells and bovine brain clathrin-coated vesicles express the mature 150-165 kDa and 130 kDa unglycosylated forms of CFTR. The localization of CFTR to brain regions controlling homeostasis and energy expenditure may relate to the pathogenesis of non-pulmonary manifestations of cystic fibrosis. CFTR expression in neurons and coated vesicles suggests a possible effect on neuropeptide vesicle trafficking by mutant CFTR.