The Golgi matrix protein giantin is required for normal cilia function in zebrafish.
The Golgi matrix protein giantin is required for normal cilia function in zebrafish.
复制标题
斑马鱼中正常的纤毛功能需要Golgi基质蛋白巨蛋白。
DOI：
10.1242/bio.025502
复制
发表时间：
2017-08-15
期刊：
影响因子：
2.4
通讯作者：
Hammond CL
中科院分区：
文献类型：
--
作者：
Bergen DJM;Stevenson NL;Skinner REH;Stephens DJ;Hammond CL
高尔基体对于新合成蛋白质（包括几乎所有细胞表面和细胞外基质蛋白聚糖）的糖基化至关重要。 Giantin 由 golgb1 基因编码，是驻留在高尔基体堆栈中的高尔金蛋白家族的成员，但其功能仍然难以捉摸。大鼠巨蛋白功能丧失会导致骨软骨发育不良；基因敲除小鼠表现出较轻微的缺陷，尤其是腭裂。在体外，巨人蛋白与高尔基体组织、生物合成运输和纤毛发生有关。在这里，我们表明，使用吗啉或敲除技术，斑马鱼中巨蛋白功能的丧失会导致纤毛功能缺陷。 Giantin morphants在神经管中的纤毛较少，而剩余的纤毛较长。突变体在神经管中具有相同数量的纤毛，但这些纤毛也被拉长。扫描电子显微镜显示巨蛋白的缺失会导致纤毛尖端物质的积累，这与逆行鞭毛内运输功能的丧失一致。突变体比变形体表现出更轻微的缺陷，这与对巨蛋白丧失的适应一致。摘要：Golgb1/giantin 的吗啡啉敲低会导致严重的纤毛病表型，并伴有更长、畸形的纤毛。稳定突变体具有非常温和的表型，表明补偿，但仍然具有更长的纤毛。
The Golgi is essential for glycosylation of newly synthesised proteins including almost all cell-surface and extracellular matrix proteoglycans. Giantin, encoded by the golgb1 gene, is a member of the golgin family of proteins that reside within the Golgi stack, but its function remains elusive. Loss of function of giantin in rats causes osteochondrodysplasia; knockout mice show milder defects, notably a cleft palate. In vitro, giantin has been implicated in Golgi organisation, biosynthetic trafficking, and ciliogenesis. Here we show that loss of function of giantin in zebrafish, using either morpholino or knockout techniques, causes defects in cilia function. Giantin morphants have fewer cilia in the neural tube and those remaining are longer. Mutants have the same number of cilia in the neural tube but these cilia are also elongated. Scanning electron microscopy shows that loss of giantin results in an accumulation of material at the ciliary tip, consistent with a loss of function of retrograde intraflagellar transport. Mutants show milder defects than morphants consistent with adaptation to loss of giantin. Summary: Morpholino knockdown of Golgb1/giantin leads to a severe cilopathy phenotype twinned with longer, misshapen cilia. Stable mutants have a very mild phenotype, indicative of compensation, but still have longer cilia.
登录
查看更多内容
影响因子：
4.4
作者：
Krock BL;Mills-Henry I;Perkins BD
通讯作者：
Perkins BD
影响因子：
4.6
作者：
Sedykh I;TeSlaa JJ;Tatarsky RL;Keller AN;Toops KA;Lakkaraju A;Nyholm MK;Wolman MA;Grinblat Y
通讯作者：
Grinblat Y
TFG促进了过渡性和有效胶原蛋白分泌的组织。
影响因子：
8.8
作者：
McCaughey J;Miller VJ;Stevenson NL;Brown AK;Budnik A;Heesom KJ;Alibhai D;Stephens DJ
通讯作者：
Stephens DJ
影响因子：
14.9
作者：
De Souza AT;Dai X;Spencer AG;Reppen T;Menzie A;Roesch PL;He Y;Caguyong MJ;Bloomer S;Herweijer H;Wolff JA;Hagstrom JE;Lewis DL;Linsley PS;Ulrich RG
通讯作者：
Ulrich RG
影响因子：
4
作者：
Asante, David;MacCarthy-Morrogh, Lucy;Stephens, David J.
通讯作者：
Stephens, David J.