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Hairy and enhancer of split 7

This gene encodes a member of the hairy and enhancer of split family of bHLH transcription factors. The mouse ortholog of this gene is regulated by Notch signaling. The protein functions as a transcriptional repressor, and is implicated in correct patterning of the axial skeleton. A mutation in this gene has been shown to result in spondylocostal dysostosis. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Sep 2009] (from NCBI)
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Top mentioned proteins: CLOCK, Lfng, galectin-1, CAN, Mesp2
Papers on Hes7
Compound heterozygous mutations in RIPPLY2 associated with vertebral segmentation defects.
Duncan et al., Australia. In Hum Mol Genet, Apr 2015
In humans, mutations in several genes involved in the Notch pathway are associated with SDV, with both autosomal recessive (MESP2, DLL3, LFNG, HES7) and autosomal dominant (TBX6) inheritance.
Canine disorder mirrors human disease: exonic deletion in HES7 causes autosomal recessive spondylocostal dysostosis in miniature Schnauzer dogs.
Wade et al., Sydney, Australia. In Plos One, Dec 2014
Mutations in the Notch signalling pathway genes DLL3, MESP2, LFNG, HES7 and TBX6 have been associated with this defect.
Whole exome sequencing in an Indian family links Coats plus syndrome and dextrocardia with a homozygous novel CTC1 and a rare HES7 variation.
Faruq et al., Bengaluru, India. In Bmc Med Genet, Dec 2014
A novel homozygous missense mutation c.1451A > C (p.H484P) in exon 9 of the CTC1 gene and a rare 3'UTR known dbSNP variation (c.*556 T > C) in HES7 were identified as the plausible candidates associated with this complex phenotype of Coats plus and dextrocardia.
The roles and mechanism of ultradian oscillatory expression of the mouse Hes genes.
Kageyama et al., Kyoto, Japan. In Semin Cell Dev Biol, Oct 2014
This periodic event is regulated by a biological clock called the segmentation clock, which involves cyclic expression of the basic helix-loop-helix gene Hes7.
The transcriptome of utricle hair cell regeneration in the avian inner ear.
Lovett et al., Saint Louis, United States. In J Neurosci, Apr 2014
For example, we show that HES7 is specifically expressed during utricle hair cell regeneration and closely parallels the expression of HES5.
3'-UTR-dependent regulation of mRNA turnover is critical for differential distribution patterns of cyclic gene mRNAs.
Bessho et al., Nara, Japan. In Febs J, 2014
Somite segmentation, a prominent periodic event in the development of vertebrates, is instructed by cyclic expression of several genes, including Hes7 and Lunatic fringe (Lfng).
Expression dynamics and functions of Hes factors in development and diseases.
Kageyama et al., Kyoto, Japan. In Curr Top Dev Biol, 2013
Hes1, Hes5, and Hes7 are known as downstream effectors of canonical Notch signaling, which regulates cell differentiation via cell-cell interaction.
Hes7 3'UTR is required for somite segmentation function.
Bessho et al., Ikoma, Japan. In Sci Rep, 2013
We attempted to elongate the oscillation period by increasing the time to transcribe Hes7 in this research.
Visualization of Notch signaling oscillation in cells and tissues.
Kageyama et al., Kyoto, Japan. In Methods Mol Biol, 2013
The Notch signaling effectors Hes1 and Hes7 exhibit oscillatory expression with a period of about 2-3 h during embryogenesis.
Higher frequency of intron loss from the promoter proximally paused genes of Drosophila melanogaster.
Niu et al., Beijing, China. In Fly (austin), 2013
Referring to previous studies on the rates of transcription and splicing and to the time saved by deletion of the introns from mouse gene Hes7, it is here suggested that transcription delay is comparable to splicing delay only when the intron is 28.5 kb or larger, which is greater in size than 95% of vertebrate introns, 99.5% of Drosophila introns, and all the annotated introns of Saccharomyces cerevisiae and Arabidopsis thaliana.
Oscillatory links of Fgf signaling and Hes7 in the segmentation clock.
Kageyama et al., Kyoto, Japan. In Curr Opin Genet Dev, 2013
Somitogenesis is controlled by the segmentation clock, where the oscillatory expression of cyclic genes such as Hes7 leads to the periodic expression of Mesp2, a master gene for somite formation.
The precise timeline of transcriptional regulation reveals causation in mouse somitogenesis network.
Kudlicki et al., Galveston, United States. In Bmc Dev Biol, 2012
Our results also indicate the presence of a new class of genes (including Raf1 and Hes7) with two peaks of activity in two distinct phases of the somite cycle.
Oscillatory gene expression and somitogenesis.
Harima et al., Kyoto, Japan. In Wiley Interdiscip Rev Dev Biol, 2012
Expression of her1 and her7 in zebrafish and Hes7 in mice oscillates by negative feedback, and mathematical models have been used to generate and test hypotheses to aide elucidation of the role of negative feedback in regulating oscillatory expression.
Mutation analysis of MESP2, HES7 and DUSP6 gene exons in patients with congenital scoliosis.
Qiu et al., Nanjing, China. In Stud Health Technol Inform, 2011
MESP2, HES7 and DUSP6 genes may not be involved in the etiopathogenesis of sporadic and non-syndromic CS in Chinese Han population.
Intronic delay is essential for oscillatory expression in the segmentation clock.
Kageyama et al., Kyoto, Japan. In Proc Natl Acad Sci U S A, 2011
introns lead to an approximately 19-min delay in the Hes7 gene expression, and mathematical modeling suggested that without such a delay, Hes7 oscillations would be abolished
Two novel missense mutations in HAIRY-AND-ENHANCER-OF-SPLIT-7 in a family with spondylocostal dysostosis.
Dunwoodie et al., Sydney, Australia. In Eur J Hum Genet, 2010
Two new missense mutations in HES7 in a family with spondylocostal dysostosis.
Sprouty4, an FGF inhibitor, displays cyclic gene expression under the control of the notch segmentation clock in the mouse PSM.
Bessho et al., Ikoma, Japan. In Plos One, 2008
Sprouty4 does not oscillate in Hes7-null mutant mouse embryos, and Hes7 can inhibit FGF-induced transcriptional activity of the Sprouty4 promoter.
Differential axial requirements for lunatic fringe and Hes7 transcription during mouse somitogenesis.
Ish-Horowicz et al., London, United Kingdom. In Plos One, 2008
there are differential axial requirements for lunatic fringe and Hes7 transcription during mouse somitogenesis
Mesp-family genes are required for segmental patterning and segmental border formation.
Takahashi et al., Mishima, Japan. In Adv Exp Med Biol, 2007
In the posterior PSM, a basic-HLH protein Hes7 plays a central role to generate traveling wave of gene expression by negatively regulating the transcription of the target genes, which may lead defining soimte spacing and future segmental unit.
Instability of Hes7 protein is crucial for the somite segmentation clock.
Kageyama et al., Kyoto, Japan. In Nat Genet, 2004
instability of Hes7 is essential for sustained oscillation and for its function as a segmentation clock
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