Abstract:
BACKGROUND INFORMATION:The BOR (branchio-oto-renal) syndrome is a dominant disorder most commonly caused by mutations in the EYA1 (Eyes Absent 1) gene. Symptoms commonly include deafness and renal anomalies. RESULTS:We have used the embryos of the frog Xenopus laevis as an animal model for early ear development to examine the effects of different EYA1 mutations. Four eya1 mRNAs encoding proteins correlated with congenital anomalies in human were injected into early stage embryos. We show that the expression of mutations associated with BOR, even in the presence of normal levels of endogenous eya1 mRNA, leads to morphologically abnormal ear development as measured by overall otic vesicle size, establishment of sensory tissue and otic innervation. The molecular consequences of mutant eya1 expression were assessed by QPCR (quantitative PCR) analysis and in situ hybridization. Embryos expressing mutant eya1 showed altered levels of multiple genes (six1, dach, neuroD, ngnr-1 and nt3) important for normal ear development. CONCLUSIONS:These studies lend support to the hypothesis that dominant-negative effects of EYA1 mutations may have a role in the pathogenesis of BOR.
journal_name
Biol Celljournal_title
Biology of the cellauthors
Li Y,Manaligod JM,Weeks DLdoi
10.1042/BC20090098subject
Has Abstractpub_date
2010-02-17 00:00:00pages
277-92issue
5eissn
0248-4900issn
1768-322Xpii
BC20090098journal_volume
102pub_type
杂志文章abstract::An interaction between human poly(A)-binding protein (PABP) et human eRF3 has been demonstrated using a double-hybrid approach and in vitro assays. Here, we show that the binding of both proteins is conserved through evolution. We also demonstrate that the last 39 C-terminal amino acids of PABP contain the interface t...
journal_title:Biology of the cell
pub_type: 杂志文章
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journal_title:Biology of the cell
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journal_title:Biology of the cell
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journal_title:Biology of the cell
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journal_title:Biology of the cell
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doi:
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journal_title:Biology of the cell
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journal_title:Biology of the cell
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journal_title:Biology of the cell
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journal_title:Biology of the cell
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journal_title:Biology of the cell
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更新日期:2004-06-01 00:00:00
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journal_title:Biology of the cell
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journal_title:Biology of the cell
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journal_title:Biology of the cell
pub_type: 杂志文章
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journal_title:Biology of the cell
pub_type: 杂志文章
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更新日期:1995-01-01 00:00:00
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journal_title:Biology of the cell
pub_type: 杂志文章,评审
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更新日期:2003-05-01 00:00:00
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journal_title:Biology of the cell
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journal_title:Biology of the cell
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journal_title:Biology of the cell
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doi:
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journal_title:Biology of the cell
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更新日期:2007-08-01 00:00:00
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journal_title:Biology of the cell
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doi:10.1111/boc.201500058
更新日期:2016-04-01 00:00:00
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journal_title:Biology of the cell
pub_type: 杂志文章,评审
doi:
更新日期:1998-10-01 00:00:00
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journal_title:Biology of the cell
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更新日期:2013-10-01 00:00:00
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journal_title:Biology of the cell
pub_type: 杂志文章
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更新日期:1990-01-01 00:00:00
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journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1111/j.1768-322x.1985.tb00375.x
更新日期:1985-01-01 00:00:00
abstract::The ultrastructure of the Drosophila subobscura chromosome regions around the breakpoints of the complex E1 + 2 + 9 + 12 gene arrangement was analyzed. This overlapping inversion is formed by the association of the E1, E2, E9, and E12 simple inversions. Ultrastructure of sections involving 58D/59A, 61C/D, 62D/63A, 64B...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1016/0248-4900(90)90359-b
更新日期:1990-01-01 00:00:00