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En-2 同源框基因靶向缺失纯合子小鼠的轻微小脑表型

Subtle cerebellar phenotype in mice homozygous for a targeted deletion of the En-2 homeobox.

作者信息

Joyner A L, Herrup K, Auerbach B A, Davis C A, Rossant J

机构信息

Samuel Lunenfeld Research Institute, Mount Sinai Hospital, Toronto, Ontario, Canada.

出版信息

Science. 1991 Mar 8;251(4998):1239-43. doi: 10.1126/science.1672471.

DOI:10.1126/science.1672471
PMID:1672471
Abstract

The two mouse genes, En-1 and En-2, that are homologs of the Drosophila segmentation gene engrailed, show overlapping spatially restricted patterns of expression in the neural tube during embryogenesis, suggestive of a role in regional specification. Mice homozygous for a targeted mutation that deletes the homeobox were viable and showed no obvious defects in embryonic development. This may be due to functional redundancy of En-2 and the related En-1 gene product during embryogenesis. Consistent with this hypothesis, the mutant mice showed abnormal foliation in the adult cerebellum, where En-2, and not En-1, is normally expressed.

摘要

两个与果蝇分节基因engrailed同源的小鼠基因En-1和En-2,在胚胎发生过程中神经管内呈现出空间限制的重叠表达模式,提示其在区域特化中发挥作用。缺失同源框的靶向突变纯合小鼠是存活的,并且在胚胎发育过程中未表现出明显缺陷。这可能是由于胚胎发生过程中En-2与相关的En-1基因产物存在功能冗余。与该假设一致,突变小鼠成年小脑出现叶状异常,正常情况下En-2而非En-1在小脑中表达。

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Subtle cerebellar phenotype in mice homozygous for a targeted deletion of the En-2 homeobox.En-2 同源框基因靶向缺失纯合子小鼠的轻微小脑表型
Science. 1991 Mar 8;251(4998):1239-43. doi: 10.1126/science.1672471.
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Abnormal embryonic cerebellar development and patterning of postnatal foliation in two mouse Engrailed-2 mutants.两种小鼠Engrailed-2突变体中胚胎期小脑发育异常及出生后叶状结构模式异常。
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The Engrailed homeobox genes determine the different foliation patterns in the vermis and hemispheres of the mammalian cerebellum.Engrailed 同源盒基因决定了哺乳动物小脑蚓部和半球的不同叶片模式。
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Rescue of the En-1 mutant phenotype by replacement of En-1 with En-2.
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Pattern deformities and cell loss in Engrailed-2 mutant mice suggest two separate patterning events during cerebellar development.Engrailed-2突变小鼠中的模式畸形和细胞丢失表明在小脑发育过程中有两个独立的模式形成事件。
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Antisense inhibition of engrailed genes in mouse embryos reveals roles for these genes in craniofacial and neural tube development.对小鼠胚胎中engrailed基因的反义抑制揭示了这些基因在颅面和神经管发育中的作用。
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