@entity50097 ( @entity70486 ) maps to the @entity19 chromosome 11 region syntenic to the common deletion interval for the @entity87 , a contiguous gene deletion syndrome. It encodes the third subunit of an eight-subunit protein complex, the COP9 signalosome (CSN), which controls a wide variety of molecules of different functions. Mutants of this complex caused lethality at early development of both plants and @entity238 . CSN function in vivo in mammals is unknown. We disrupted the @entity19 @entity50097 gene in three independent ways with insertional vectors, including constructing a approximately 3-Mb inversion chromosome. The heterozygous @entity19 appeared normal, although the protein level was reduced. @entity50097 (-/-) @entity1167 after 5.5 days postcoitum (dpc) and resorbed by 8.5 dpc. Mutant embryos form an abnormal egg cylinder which does not gastrulate. They have reduced numbers of epiblast cells, mainly due to increased cell death. In the @entity50097 (-/-) @entity19 , subunit 8 of the COP9 complex was not detected by immunohistochemical techniques, suggesting that the absence of @entity50097 may disrupt the entire COP9 complex. Therefore, @entity50097 is important for maintaining the integrity of the COP9 signalosome and is crucial to maintain the survival of epiblast cells and thus the development of the postimplantation embryo in @entity19 .

XXXX is essential for maintenance of cell proliferation in the @entity19 embryonic epiblast.

@entity70486 :: ('26572(Tax:10090)', 'Gene') :: ['Cops3'] @entity87 :: ('MESH:D058496', 'Disease') :: ['Smith-Magenis syndrome'] @entity19 :: ('10090', 'Species') :: ['mice', 'mouse', 'murine'] @entity50097 :: ('12994(Tax:10090)', 'Gene') :: ['Csn3'] @entity1167 :: ('MESH:D006323', 'Disease') :: ['embryos arrested'] @entity238 :: ('7227', 'Species') :: ['Drosophila melanogaster']
@entity70486 :: (26572(Tax:10090),Gene) :: ['COP9 signalosome subunit 3']
