ArticleThe Journal of biological chemistry2008
Molecular consequences of a frameshifted DLX3 mutant leading to Tricho-Dento-Osseous syndrome.
Article in The Journal of biological chemistry, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 45 citations in OpenAlex.
- A novel EDA variant that causes X-linked hypohidrotic ectodermal dysplasia in a Chinese family.BMC pregnancy and childbirth · 2025Article
- MAST4 regulates stem cell maintenance with DLX3 for epithelial development and amelogenesis.Experimental & molecular medicine · 2024Article
- Trafficking and secretion of keratin 75 by ameloblastsThe Journal of biological chemistry · 2019Article
- Novel DLX3 variants in amelogenesis imperfecta with attenuated tricho-dento-osseous syndrome.Oral diseases · 2019Article
- Anthropoid primate-specific retroviral element THE1B controls expression of CRH in placenta and alters gestation length.PLoS biology · 2018Article
- DLX3-Dependent STAT3 Signaling in Keratinocytes Regulates Skin Immune Homeostasis.The Journal of investigative dermatology · 2018Article
- Homeobox transcription factor DLX4 is not necessary for skin development and homeostasis.Experimental dermatology · 2018Article
- Genetic variants in pachyonychia congenita-associated keratins increase susceptibility to tooth decay.PLoS genetics · 2018Article
- BMP-2 induced Dspp transcription is mediated by Dlx3/Osx signaling pathway in odontoblasts.Scientific reports · 2017Article
- A novel DLX3-PKC integrated signaling network drives keratinocyte differentiation.Cell death and differentiation · 2017Article
- DLX3-Dependent Regulation of Ion Transporters and Carbonic Anhydrases is Crucial for Enamel Mineralization.Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research · 2017Article
- Amelogenesis Imperfecta; Genes, Proteins, and Pathways.Frontiers in physiology · 2017Review
- The homeoprotein DLX3 and tumor suppressor p53 co-regulate cell cycle progression and squamous tumor growth.Oncogene · 2016Article
- MicroRNA 665 Regulates Dentinogenesis through MicroRNA-Mediated Silencing and Epigenetic Mechanisms.Molecular and cellular biology · 2015Article
- Bmp2 deletion causes an amelogenesis imperfecta phenotype via regulating enamel gene expression.Journal of cellular physiology · 2015Article
- Transcriptional factor DLX3 promotes the gene expression of enamel matrix proteins during amelogenesis.PloS one · 2015Article
- S100A14, a member of the EF-hand calcium-binding proteins, is overexpressed in breast cancer and acts as a modulator of HER2 signaling.The Journal of biological chemistry · 2014Article
- Immortalized mouse dental papilla mesenchymal cells preserve odontoblastic phenotype and respond to bone morphogenetic protein 2.In vitro cellular & developmental biology. Animal · 2013Article
- The transcription factor DLX3 regulates the osteogenic differentiation of human dental follicle precursor cells.Stem cells and development · 2012Article
- Neural crest deletion of Dlx3 leads to major dentin defects through down-regulation of Dspp.The Journal of biological chemistry · 2012Article
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Authors and funding
7 authors at 4 institutions in 2 countries.
Funding
Abstract
The homeodomain protein Distal-less-3 (Dlx3) plays a crucial role during embryonic development. This transcription factor is known to be essential for placental formation and to be involved in skin and skeletal organogenesis. In humans, a frameshift mutation in the coding sequence of the DLX3 gene results in an ectodermal dysplasia called Tricho-Dento-Osseous syndrome (TDO). The main features of this autosomal dominant disorder are defects in hair, teeth, and bone. To investigate the functional alterations caused by the mutated DLX3(TDO) isoform ex vivo, we used tetracycline-inducible osteoblastic and keratinocyte cell lines and calvarial derived osteoblasts in which the expression of DLX3(WT) and/or DLX3(TDO) could be regulated and monitored. Immunocytochemical analysis revealed that both DLX3(WT) and DLX3(TDO) recombinant proteins are targeted to the nucleus. However, as demonstrated by electrophoresis mobility shift assay, DLX3(TDO) is not able to bind to the canonical Dlx3 binding site. Furthermore, we demonstrate that the frameshifted C-terminal domain in DLX3(TDO) is accountable for the loss of DNA binding activity because the C-terminal domain in DLX3(WT) is not required for DNA binding activity. Although DLX3(TDO) alone cannot bind to a Dlx3 responsive element, when DLX3(WT) and DLX3(TDO) are co-expressed they form a complex that can bind DNA. Concomitant with the inability to bind DNA, DLX3(TDO) has a defective transcriptional activity. Moreover, the transcriptional activity of DLX3(WT) is significantly reduced in the presence of the mutated isoform, indicating that DLX3(TDO) has a dominant negative effect on DLX3(WT) transcriptional activity.
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