Mohamed H Al-Hamed,Charles van Lennep,Ann Marie Hynes et al.
Mohamed H Al-Hamed et al.
Background: Bardet-Biedl syndrome (BBS) is an autosomal recessive ciliopathy disorder with 18 known causative genes (BBS1-18). The primary clinical features are renal abnormalities, rod-cone dystrophy, post-axial polydact...
Reduction of meckelin leads to general loss of cilia, ciliary microtubule misalignment and distorted cell surface organization [0.03%]
Meckelin的减少导致纤毛丢失、微管排列错乱及细胞表面结构扭曲
Tyler Picariello,Megan Smith Valentine,Junji Yano et al.
Tyler Picariello et al.
Background: Meckelin (MKS3), a conserved protein linked to Meckel Syndrome, assists in the migration of centrioles to the cell surface for ciliogenesis. We explored for additional functions of MKS3p using RNA interference...
Enhanced response to pulmonary Streptococcus pneumoniae infection is associated with primary ciliary dyskinesia in mice lacking Pcdp1 and Spef2 [0.03%]
缺乏Pcdp1和Spef2的小鼠主要纤毛运动障碍对肺部肺炎链球菌感染的反应增强
Casey W McKenzie,Joshua M Klonoski,Taylor Maier et al.
Casey W McKenzie et al.
Background: Lower airway abnormalities are common in patients with primary ciliary dyskinesia (PCD), a pediatric syndrome that results from structural or functional defects in motile cilia. Patients can suffer from recurr...
Interleukin-1β sequesters hypoxia inducible factor 2α to the primary cilium [0.03%]
缺氧诱导因子2α在细胞初级纤毛中的定位受白介素-1β调控
Angus Kt Wann,Clare L Thompson,J Paul Chapple et al.
Angus Kt Wann et al.
Background: The primary cilium coordinates signalling in development, health and disease. Previously we have shown that the cilium is essential for the anabolic response to loading and the inflammatory response to interle...
Getting to the heart of intraflagellar transport using Trypanosoma and Chlamydomonas models: the strength is in their differences [0.03%]
用巴贝斯虫和绿藻模型研究鞭毛内运输到达核心：差异产生优势
Benjamin Morga,Philippe Bastin
Benjamin Morga
Cilia and flagella perform diverse roles in motility and sensory perception, and defects in their construction or their function are responsible for human genetic diseases termed ciliopathies. Cilia and flagella construction relies on intra...
Karl-Ferdinand Lechtreck,Travis J Gould,George B Witman
Karl-Ferdinand Lechtreck
Background: Most motile cilia and flagella have nine outer doublet and two central pair (CP) microtubules. Outer doublet microtubules are continuous with the triplet microtubules of the basal body, are templated by the ba...
New mutations in flagellar motors identified by whole genome sequencing in Chlamydomonas [0.03%]
利用整个基因组测序在绿藻中鉴定出鞭毛马达的新突变体
Huawen Lin,Nicholas P Nauman,Alison J Albee et al.
Huawen Lin et al.
Background: The building of a cilium or flagellum requires molecular motors and associated proteins that allow the relocation of proteins from the cell body to the distal end and the return of proteins to the cell body in...
Ciliogenesis and cerebrospinal fluid flow in the developing Xenopus brain are regulated by foxj1 [0.03%]
Foxj1调控非洲爪蟾脑发育过程中的纤毛发生及脑脊液流动
Cathrin Hagenlocher,Peter Walentek,Christina M Ller et al.
Cathrin Hagenlocher et al.
Background: Circulation of cerebrospinal fluid (CSF) through the ventricular system is driven by motile cilia on ependymal cells of the brain. Disturbed ciliary motility induces the formation of hydrocephalus, a pathologi...
The Small GTPase Rsg1 is important for the cytoplasmic localization and axonemal dynamics of intraflagellar transport proteins [0.03%]
小GTP酶RSG1对于逆向动力蛋白复合体和内共生体运输蛋白胞质定位及纤毛动态变化具有重要作用
Eric R Brooks,John B Wallingford
Eric R Brooks
Background: Cilia are small, microtubule-based protrusions important for development and homeostasis. We recently demonstrated that the planar cell polarity effector protein Fuz is a critical regulator of axonemal intrafl...
William Y Tsang,Brian D Dynlacht
William Y Tsang
Cilia are hair-like protrusions found at the surface of most eukaryotic cells. They can be divided into two types, motile and non-motile. Motile cilia are found in a restricted number of cell types, are generally present in large numbers, a...