新型的中心角缺陷在缺少腺酸激酶7的状上皮质的初级状失动现象型的基础上
Jennifer Sheridan1, Aline Grata1, Eve E Suva1
1Northwestern University, Feinberg School of Medicine, Department of Cell and Developmental Biology.
bioRxiv : the preprint server for biology
|August 7, 2023
概括
腺酸酶7 (AK7) 影响中心球的形成和功能,这对于运动的细胞至关重要. 在Xenopus胚胎中,AK7的枯竭会损害毛发育和粘膜流,从而提供了对初级毛功能障碍 (PCD) 的见解.
科学领域:
- 细胞生物学 细胞生物学
- 发展生物学 发展生物学
- 遗传学 遗传学 是一个
背景情况:
- 在Xenopus胚胎中的多细胞 (MCC) 产生流体流,这对于粘膜分布至关重要.
- 纤毛功能障碍导致人类的初级纤毛功能障碍 (PCD),与许多基因的突变有关.
- 低基因酶7 (AK7) 基因表达和AK7突变与PCD有关,但机制尚不清楚.
研究的目的:
- 调查基酶7 (AK7) 在毛发育和功能中的作用.
- 阐明AK7通过哪些细胞机制影响纤毛发育和粘膜流.
- 建立一个Xenopus模型来研究AK7相关的PCD表型.
主要方法:
- 在Xenopus胚胎中分析了AK7基因表达和蛋白质定位.
- 操纵AK7水平 (过度表达和耗尽) 以评估对MCCs的影响.
- 的数量,长度,跳动频率和组织范围内的粘膜流量都被量化了.
- 使用显微镜检查了心心点数和定位.
主要成果:
- AK7以DPY30域依赖的方式局部化到毛,这表明毛功能.
- AK7的过度表达增加了中心点数,这表明在中心点生物发生过程中发挥了作用.
- AK7枯竭减少了乳毛的数量,长度和跳动频率,损害了粘膜细胞的流动.
- AK7 枯竭还降低了中心点数和增加了亚顶点中心点数,这表明它在生物发生和对接中发挥了作用.
结论:
- AK7对于正确的纤维生成至关重要,因为它会影响中心球生物生成和顶端对接.
- AK7功能的缺陷导致毛功能减弱和粘膜膜清除受损,符合PCD.
- 这项研究为了解AK7在PCD病变发生过程中的作用提供了细胞基础.
相关概念视频
Microtubules in Signaling
1.7K
The primary cilium, made up of microtubules, acts as antennae on the cell surfaces for relaying external stimuli into the cells. These fine hair-like structures are present, generally one per cell. These are non-motile cilia in a 9+0 microtubules arrangement, where the central pair of microtubules are absent. The primary cilia arise from the basal body embedded in the cell membrane. Intraflagellar transport (IFT) carries requisite proteins from the cytoplasm to the cilium because the primary...
1.7K
Mechanism of Ciliary Motion
3.7K
The ciliary structures were first seen in 1647 by Antonie Leeuwenhoek while observing the protozoans. In lower organisms, these appendages are responsible for cell movement, while in higher organisms, these appendages help in the movement of the extracellular fluids within the body cavities.
The cilia are made up of microtubules in a 9+2 arrangement, with nine microtubule doublet ring bundles, surrounding a pair of central singlet microtubule bundles. The doublet microtubule bundles are...
The cilia are made up of microtubules in a 9+2 arrangement, with nine microtubule doublet ring bundles, surrounding a pair of central singlet microtubule bundles. The doublet microtubule bundles are...
3.7K
Centrosome Duplication
4.1K
The primary microtubule organizing center (MTOC) in animal cells is the centrosome. A centrosome has two cylindrical centrioles at its core. Each centriole consists of nine sets of three microtubules held together by proteins. The centrioles are positioned at right angles to each other and surrounded by a shapeless protein cloud called the pericentriolar matrix, or pericentriolar material (PCM).
To ensure that each daughter cell receives a centrosome after cell division, centrosome duplication...
To ensure that each daughter cell receives a centrosome after cell division, centrosome duplication...
4.1K
Centrioles and Centrosomes
2.8K
Most animal cells comprise a pair of centrioles together called a centrosome. The cell duplicates its centrosome and contains two centrosomes side-by-side, which begin to move apart during the prophase. As the centrosomes migrate to two different sides of the cell, microtubules start extending from each centrosome toward the other end. The mitotic spindle is composed of the centrosomes and their emerging microtubules.
Near the end of the prophase, also called late prophase or...
Near the end of the prophase, also called late prophase or...
2.8K
Microtubules in Cell Motility
3.3K
Microtubules are thick hollow cylindrical proteins that help form the cytoskeleton. Microtubules have varied roles in the cell. These filaments help form cellular appendages like cilia and flagella, which are responsible for locomotion. The cilia arise from basal bodies, separated from the main body by a membrane-like structure forming the transition zone. This zone is the gate for the entry of lipids and proteins, creating a unique composition of lipids and proteins in the ciliary membrane and...
3.3K
Cystic Fibrosis: Pathogenesis
281
Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
281


