Planar cell polarity genes Celsr1 and Vangl2 are necessary for kidney growth, differentiation, and rostrocaudal patterning.
Planar cell polarity genes Celsr1 and Vangl2 are necessary for kidney growth, differentiation, and rostrocaudal patterning.
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DOI：
10.1016/j.kint.2016.07.011
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发表时间：
2016-12
影响因子：
19.6
通讯作者：
Long, David A.
中科院分区：
文献类型：
--
作者：
Brzoska, Hortensja L.;d'Esposito, Angela M.;Kolatsi-Joannou, Maria;Patel, Vishal;Igarashi, Peter;Lei, Yunping;Finnell, Richard H.;Lythgoe, Mark F.;Woolf, Adrian S.;Papakrivopoulou, Eugenia;Long, David A.
关键词：
哺乳动物的肾脏含有肾小球和与输尿管芽状集合管相连的小管。它有一个典型的豆状形状，在门部周围有近乎完全的吻尾对称。在这里，我们证明了Celsr1，一个与神经管形态发生有关的平面细胞极性(PCP)基因，是输尿管树生长所必需的，在发育早期和妊娠后期防止小管过度生长。我们还发现Celsr1和Vangl2(另一个PCP基因)在输尿管树生长过程中存在相互作用，最明显的是在复合杂合突变小鼠的肾脏尾部，这些小鼠的臀部发育迟缓。此外，这些基因共同是肾小球成熟所必需的。有趣的是，我们证明了CELSR1突变和脊柱裂的患者可以有显著的肾脏畸形。因此，PCP基因在哺乳动物肾脏发育中是重要的，并在器官发生过程中对吻尾模式具有意想不到的作用。
The mammalian kidney contains nephrons comprising glomeruli and tubules joined to ureteric bud–derived collecting ducts. It has a characteristic bean-like shape, with near-complete rostrocaudal symmetry around the hilum. Here we show that Celsr1, a planar cell polarity (PCP) gene implicated in neural tube morphogenesis, is required for ureteric tree growth in early development and later in gestation prevents tubule overgrowth. We also found an interaction between Celsr1 and Vangl2 (another PCP gene) in ureteric tree growth, most marked in the caudal compartment of the kidneys from compound heterozygous mutant mice with a stunted rump. Furthermore, these genes together are required for the maturation of glomeruli. Interestingly, we demonstrated patients with CELSR1 mutations and spina bifida can have significant renal malformations. Thus, PCP genes are important in mammalian kidney development and have an unexpected role in rostrocaudal patterning during organogenesis.
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