“重新发现”了一个被遗忘的细胞器,初级纤毛:许多疾病的根本原因

D. Wheatley
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引用次数: 2

摘要

初级纤毛是在19世纪后期被发现的。其存在的确凿证据需要电子显微镜的出现(20世纪50年代至60年代),之后由Sorokin将其与(9 + 2)品种的活动纤毛进行比较。尽管从这一时期到20世纪90年代末,只有一小群研究人员对初级纤毛进行了研究,但由于它在细胞内信号传导中产生ca2 +瞬态,它作为传感器的功能(之前由Tony Poole提倡)得以确立。纤毛发育不全或功能障碍的病理生物学后果在20世纪90年代中期得到强调。但直到几年后认识到发育不全可能是由于鞭毛内转运蛋白的突变引起的,病理后遗症才得到认识。自21世纪初以来,初级纤毛已经被认为在细胞行为和发育中具有许多功能,因此,在身体的许多组织中,这种几乎无处不在的细胞器的紊乱会导致从多囊肾病到阿尔茨海默氏症的惊人广泛的症状。一个多世纪以来,这种细胞器被大多数细胞生物学家视为退化或初级,但在几乎任何医学和发育条件下都不能再被忽视。关于这个迷人的细胞器的生物学,还有很多东西需要学习。生物医学评论2013;24: 1 - 7。
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"Rediscovery" of a forgotten organelle, the primary cilium: the root cause of a plethora of disorders
The primary cilium was recognised in the late 19th century. Conclusive evidence of its existence required the advent of the electron microscope (1950s-1960s), after which its comparison with motile cilia of the (9 + 2) variety was made by Sorokin. Although a small group of devotees researched the primary cilium from this period until the late 1990s, its function as a sensor (previously advocated by Tony Poole) was established because it produced Ca 2+ transients in intracellular signalling. The pathobiological consequences of ciliary agenesis or dysfunction was emphasised in the mid 1990s. But it was only after the recognition that agenesis could be due to mutations in intraflagellar transport proteins several years later that the pathological sequelae were appreciated. Since the early 2000s, the primary cilium has now been implicated as having many functions in cellular behaviour and development, such that disorder in this almost ubiquitous organelle in many tissues of the body leads to an astonishingly wide range of symptoms, from polycystic kidney disease to Alzheimer's. This organelle, dismissed as vestigial or rudimentary by most cell biologists for well over a century, can no longer be ignored in almost any medical and development condition. There is also very much more to learn about the biology of this fascinating organelle. Biomedical Reviews 2013; 24: 1-7.
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