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纤毛长度异常——原发性纤毛运动障碍的一个病因——病例报告

Abnormal length of cilia--a cause of primary ciliary dyskinesia--a case report.

作者信息

Niggemann B, Müller A, Nolte A, Schnoy N, Wahn U

机构信息

University Childrens Hospital (KAVH), Department Paediatric Pneumology and Immunology, Berlin, Federal Republic of Germany.

出版信息

Eur J Pediatr. 1992 Jan;151(1):73-5. doi: 10.1007/BF02073899.

DOI:10.1007/BF02073899
PMID:1728552
Abstract

A 7-year-old Turkish boy had suffered from chronic coughing from early childhood. Severe bronchiectasis in the right lung was confirmed by bronchography. Ciliary beat frequency determined in a bronchial mucosal biopsy was markedly decreased (5.7 Hz). Electron microscopy revealed cilia with a length of 15 microns. No structural abnormality was found. A possible link between the abnormally long, slow beating cilia and the clinical symptoms is discussed.

摘要

一名7岁土耳其男孩自幼患有慢性咳嗽。支气管造影证实右肺有严重支气管扩张。支气管黏膜活检测定的纤毛摆动频率明显降低(5.7次/秒)。电子显微镜检查显示纤毛长度为15微米,未发现结构异常。本文讨论了异常长且摆动缓慢的纤毛与临床症状之间可能存在的联系。

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Abnormal length of cilia--a cause of primary ciliary dyskinesia--a case report.纤毛长度异常——原发性纤毛运动障碍的一个病因——病例报告
Eur J Pediatr. 1992 Jan;151(1):73-5. doi: 10.1007/BF02073899.
2
Freeze-fracture analysis of the respiratory cilia from the bronchial mucosa of a patient with primary ciliary dyskinesia.
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本文引用的文献

1
Nasal mucociliary clearance and ciliary beat frequency in cystic fibrosis compared with sinusitis and bronchiectasis.与鼻窦炎和支气管扩张症相比,囊性纤维化患者的鼻黏液纤毛清除功能和纤毛摆动频率
Thorax. 1981 Sep;36(9):654-8. doi: 10.1136/thx.36.9.654.
2
Nasal ciliary ultrastructure and function in patients with primary ciliary dyskinesia compared with that in normal subjects and in subjects with various respiratory diseases.原发性纤毛运动障碍患者的鼻纤毛超微结构和功能与正常受试者及各种呼吸系统疾病受试者的比较。
Am Rev Respir Dis. 1984 Jan;129(1):161-7. doi: 10.1164/arrd.1984.129.1.161.
3
Ciliary dyskinesia with normal ultrastructure.
肾上皮细胞中RCK激酶ICK和MOK对纤毛长度和鞭毛内运输的调控。
PLoS One. 2014 Sep 22;9(9):e108470. doi: 10.1371/journal.pone.0108470. eCollection 2014.
4
Target-of-rapamycin complex 1 (Torc1) signaling modulates cilia size and function through protein synthesis regulation.雷帕霉素靶蛋白复合物 1(Torc1)信号通过调节蛋白质合成来调节纤毛大小和功能。
Proc Natl Acad Sci U S A. 2012 Feb 7;109(6):2021-6. doi: 10.1073/pnas.1112834109. Epub 2012 Jan 23.
5
A CDK-related kinase regulates the length and assembly of flagella in Chlamydomonas.一种与细胞周期蛋白依赖性激酶(CDK)相关的激酶调节衣藻中鞭毛的长度和组装。
J Cell Biol. 2007 Mar 12;176(6):819-29. doi: 10.1083/jcb.200610022.
6
Regulation of flagellar assembly by glycogen synthase kinase 3 in Chlamydomonas reinhardtii.莱茵衣藻中糖原合酶激酶3对鞭毛组装的调控
Eukaryot Cell. 2004 Oct;3(5):1307-19. doi: 10.1128/EC.3.5.1307-1319.2004.
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Defective flagellar assembly and length regulation in LF3 null mutants in Chlamydomonas.衣藻LF3基因敲除突变体中鞭毛组装和长度调控缺陷。
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8
Kartagener syndrome: an uncommon cause of neonatal respiratory distress?
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具有正常超微结构的睫状运动障碍
Thorax. 1983 Nov;38(11):875-6. doi: 10.1136/thx.38.11.875.
4
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Schweiz Med Wochenschr. 1984 May 5;114(18):610-9.
5
Non-invasive sampling of nasal cilia for measurement of beat frequency and study of ultrastructure.用于测量摆动频率和超微结构研究的鼻纤毛无创采样。
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6
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Eur J Respir Dis. 1985 Mar;66(3):173-80.
7
Ciliary abnormalities in respiratory disease.呼吸系统疾病中的纤毛异常。
Arch Dis Child. 1988 Mar;63(3):238-43. doi: 10.1136/adc.63.3.238.
8
Freeze-fracture analysis of the respiratory cilia from the bronchial mucosa of a patient with primary ciliary dyskinesia.
Respiration. 1988;53(1):44-9. doi: 10.1159/000195395.
9
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