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首页> 外文期刊>Cellular Physiology and Biochemistry >Ceramide in pseudomonas aeruginosa infections and cystic fibrosis
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Ceramide in pseudomonas aeruginosa infections and cystic fibrosis

机译：神经酰胺在铜绿假单胞菌感染和囊性纤维化中的作用

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摘要

Cystic fibrosis is one of the most common autosomal recessive disorders, at least in Western countries. Patients with cystic fibrosis exhibit many symptoms, the most important of which are chronic inflammation and bacterial infections of the lung. Cystic fibrosis is caused by mutations of the cystic fibrosis conductance regulator (CFTR) gene; however, the molecular mechanisms leading to the clinical manifestations of cystic fibrosis are still unclear. Here we discuss recent findings related to the role of sphingolipids, in particular ceramide, in cystic fibrosis and the bacterial infections associated with that disease. Ceramide accumulates in the lungs of cystic fibrosis mice and causes pulmonary inflammation, infection, and cell death, events that are corrected by the genetic deletion or pharmacological inhibition of acid sphingomyelinase; this inhibition normalizes ceramide concentrations in murine models of cystic fibrosis. Initial clinical studies suggest that pharmacological inhibition of acid sphingomyelinase may be a novel strategy for treating patients with cystic fibrosis.
机译：囊性纤维化是最常见的常染色体隐性遗传疾病之一，至少在西方国家如此。患有囊性纤维化的患者表现出许多症状，其中最重要的是慢性炎症和肺部细菌感染。囊性纤维化是由囊性纤维化电导调节因子（CFTR）基因的突变引起的。然而，导致囊性纤维化临床表现的分子机制仍不清楚。在这里，我们讨论与鞘脂特别是神经酰胺在囊性纤维化中的作用以及与该疾病相关的细菌感染有关的最新发现。神经酰胺在囊性纤维化小鼠的肺中蓄积并引起肺部炎症，感染和细胞死亡，这些事件可通过酸性鞘磷脂酶的基因缺失或药理抑制来纠正;这种抑制作用使鼠类囊性纤维化模型中的神经酰胺浓度正常化。初步的临床研究表明，酸性鞘磷脂酶的药理抑制作用可能是治疗囊性纤维化患者的新策略。

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