24 research outputs found

    Zic2 and Zic3 synergistically control neurulation and segmentation of paraxial mesoderm in mouse embryo

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    AbstractZic family zinc-finger proteins play various roles in animal development. In mice, five Zic genes (Zic1–5) have been reported. Despite the partly overlapping expression profiles of these genes, mouse mutants for each Zic show distinct phenotypes. To uncover possible redundant roles, we characterized Zic2/Zic3 compound mutant mice. Zic2 and Zic3 are both expressed in presomitic mesoderm, forming and newly generated somites with differential spatiotemporal accentuation. Mice heterozygous for the hypomorphic Zic2 allele together with null Zic3 allele generally showed severe malformations of the axial skeleton, including asymmetric or rostro-caudally bridged vertebrae, and reduction of the number of caudal vertebral bones, that are not obvious in single mutants. These defects were preceded by perturbed somitic marker expression, and reduced paraxial mesoderm progenitors in the primitive streak. These results suggest that Zic2 and Zic3 cooperatively control the segmentation of paraxial mesoderm at multiple stages. In addition to the segmentation abnormality, the compound mutant also showed neural tube defects that ran the entire rostro-caudal extent (craniorachischisis), suggesting that neurulation is another developmental process where Zic2 and Zic3 have redundant functions

    ERAD components Derlin-1 and Derlin-2 are essential for postnatal brain development and motor function

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    Derlin family members (Derlins) are primarily known as components of the endoplasmic reticulum-associated degradation pathway that eliminates misfolded proteins. Here we report a function of Derlins in the brain development. Deletion of Derlin-1 or Derlin-2 in the central nervous system of mice impaired postnatal brain development, particularly of the cerebellum and striatum, and induced motor control deficits. Derlin-1 or Derlin-2 deficiency reduced neurite outgrowth in vitro and in vivo and surprisingly also inhibited sterol regulatory element binding protein 2 (SREBP-2)-mediated brain cholesterol biosynthesis. In addition, reduced neurite outgrowth due to Derlin-1 deficiency was rescued by SREBP-2 pathway activation. Overall, our findings demonstrate that Derlins sustain brain cholesterol biosynthesis, which is essential for appropriate postnatal brain development and function

    コウセイジョウフンイキカ デノ ネツCVDホウ ニヨル Siジョウ ノ Geセイチョウ ショキカテイ

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    The growth characteristics in the initial stage of Ge epitaxy on the Si(100) epitaxial buffer layer have been investigated by ultraclean LPCVD at 350℃ using GeH_4 with H_2 or Ar as a carrier gas. When H_2 was used as a carrier gas, an incubation period for Ge nucleus formation on Si was found. After the incubation period layer growth of Ge film started. When Ar was used as a carrier gas, the incubation period was drastically reduced without any change in the layer growth rate. The nucleus size was larger and the nucleus density was lower in the case of using H_2 as a carrier gas in comparison with using Ar. These growth characteristics are attributed to the suppression of adsorption and/or decomposition of GeH_4 on the H-terminated Si surface in the case of H_2 as a carrier gas
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