人类疾病中的纤毛结构和功能

Gregory J. Pazour
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引用次数: 0

摘要

纤毛功能障碍会导致一大类发育性和退行性人类疾病,即纤毛疾病。这些疾病反映了纤毛在感知环境和产生运动力方面的关键作用。感官功能包括视觉和嗅觉。此外,遍布全身的原生纤毛和运动纤毛还能监测环境,使细胞与周围的细胞协调其生物学特性。这种协调对器官的发育和维护至关重要,纤毛功能障碍会导致各种结构性先天缺陷和退行性疾病。纤毛运动的缺陷会导致各种健康问题:肺部疾病源于粘液纤毛清除能力受损;男性不育源于精子运动能力受损,无法有效穿过传出导管;左右轴的紊乱源于结节纤毛无法建立准确的左右线索。
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Cilia structure and function in human disease

Ciliary dysfunction causes a large group of developmental and degenerative human diseases known as ciliopathies. These diseases reflect the critical roles that cilia play in sensing the environment and in force generation for motility. Sensory functions include our senses of vision and olfaction. In addition, primary and motile cilia throughout our body monitor the environment allowing cells to coordinate their biology with the cells around them. This coordination is critical to organ development and maintenance, and ciliary dysfunction causes diverse structural birth defects and degenerative diseases. Deficiencies in motility lead to various health issues: lung diseases arise from impaired mucociliary clearance; male infertility results from compromised sperm motility and their inability to traverse the efferent ducts effectively; and disruptions in the left-right axis stem from nodal cilia's failure to establish accurate left-right cues.

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来源期刊
Current Opinion in Endocrine and Metabolic Research
Current Opinion in Endocrine and Metabolic Research Medicine-Endocrinology, Diabetes and Metabolism
CiteScore
4.10
自引率
0.00%
发文量
80
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