健康和囊性纤维化猪气道中的黏液纤毛转运。
Mucociliary Transport in Healthy and Cystic Fibrosis Pig Airways.
机构信息
1 Department of Internal Medicine.
2 Department of Pediatrics.
出版信息
Ann Am Thorac Soc. 2018 Nov;15(Suppl 3):S171-S176. doi: 10.1513/AnnalsATS.201805-308AW.
Abstract
Cystic fibrosis (CF) lung disease is the major cause of morbidity and mortality in people with CF. Abnormal mucociliary transport has been the leading hypothesis for the underlying pathogenesis of CF airway disease. However, this has been difficult to investigate at very early time points. A porcine CF model, which recapitulates many features of CF disease in humans, enables studies to be performed in non-CF and CF pigs on the day that they are born. In newborn CF pigs, we found that under basal conditions, mucociliary transport rates in non-CF and CF pigs are similar. However, after cholinergic stimulation, which stimulates submucosal gland secretion, particles become stuck in the CF airways owing to a failure of mucus strands to release from submucosal glands. In this review, we summarize these recent discoveries and also discuss the morphology, composition, and function of mucins in the porcine lung.
摘要
囊性纤维化（CF）肺部疾病是 CF 患者发病和死亡的主要原因。异常的黏液纤毛转运一直是 CF 气道疾病潜在发病机制的主要假说。然而，这在非常早期的时间点很难进行研究。一种猪 CF 模型，可在人类 CF 疾病中重现许多特征，使人们能够在非 CF 和 CF 猪出生的当天对其进行研究。在新生 CF 猪中，我们发现，在基础条件下，非 CF 和 CF 猪的黏液纤毛转运率相似。然而，在刺激黏膜下腺分泌的胆碱能刺激后，由于黏液丝未能从黏膜下腺释放，颗粒会卡在 CF 气道中。在这篇综述中，我们总结了这些最新发现，并讨论了猪肺中黏蛋白的形态、组成和功能。
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本文引用的文献
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Eur Respir J. 2018 Aug 30;52(2). doi: 10.1183/13993003.00457-2018. Print 2018 Aug.
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The normal trachea is cleaned by MUC5B mucin bundles from the submucosal glands coated with the MUC5AC mucin.正常气管由覆盖有MUC5AC粘蛋白的粘膜下腺的MUC5B粘蛋白束进行清洁。
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