Abrogation of epithelial BMP2 and BMP4 causes Amelogenesis Imperfecta by reducing MMP20 and KLK4 expression View Full Text


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Article Info

DATE

2016-07

AUTHORS

Xiaohua Xie, Chao Liu, Hua Zhang, Priyam H Jani, Yongbo Lu, Xiaofang Wang, Bin Zhang, Chunlin Qin

ABSTRACT

Amelogenesis Imperfecta (AI) can be caused by the deficiencies of enamel matrix proteins, molecules responsible for the transportation and secretion of enamel matrix components, and proteases processing enamel matrix proteins. In the present study, we discovered the double deletion of bone morphogenetic protein 2 (Bmp2) and bone morphogenetic protein 4 (Bmp4) in the dental epithelium by K14-cre resulted in hypoplastic enamel and reduced density in X-ray radiography as well as shortened enamel rods under scanning electron microscopy. Such enamel phenotype was consistent with the diagnosis of hypoplastic amelogenesis imperfecta. Histological and molecular analyses revealed that the removal of matrix proteins in the mutant enamel was drastically delayed, which was coincided with the greatly reduced expression of matrix metalloproteinase 20 (MMP20) and kallikrein 4 (KLK4). Although the expression of multiple enamel matrix proteins was down-regulated in the mutant ameloblasts, the cleavage of ameloblastin was drastically impaired. Therefore, we attributed the AI primarily to the reduction of MMP20 and KLK4. Further investigation found that BMP/Smad4 signaling pathway was down-regulated in the K14-cre;Bmp2(f/f);Bmp4(f/f)ameloblasts, suggesting that the reduced MMP20 and KLK4 expression may be due to the attenuated epithelial BMP/Smad4 signaling. More... »

PAGES

25364

References to SciGraph publications

  • 2010-09. Inherited human diseases of heterotopic bone formation in NATURE REVIEWS RHEUMATOLOGY
  • Identifiers

    URI

    http://scigraph.springernature.com/pub.10.1038/srep25364

    DOI

    http://dx.doi.org/10.1038/srep25364

    DIMENSIONS

    https://app.dimensions.ai/details/publication/pub.1039678616

    PUBMED

    https://www.ncbi.nlm.nih.gov/pubmed/27146352


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    curl -H 'Accept: application/ld+json' 'https://scigraph.springernature.com/pub.10.1038/srep25364'

    N-Triples is a line-based linked data format ideal for batch operations.

    curl -H 'Accept: application/n-triples' 'https://scigraph.springernature.com/pub.10.1038/srep25364'

    Turtle is a human-readable linked data format.

    curl -H 'Accept: text/turtle' 'https://scigraph.springernature.com/pub.10.1038/srep25364'

    RDF/XML is a standard XML format for linked data.

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    297 https://www.grid.ac/institutes/grid.416972.f schema:alternateName Texas A&M Health Science Center
    298 schema:name Department of Biomedical Sciences and Center for Craniofacial Research and Diagnosis, Texas A&M University Baylor College of Dentistry, Dallas, Texas, 75246, USA.
    299 Department of Stomatology, and Institute of Hard Tissue Development and Regeneration, the 2nd Affiliated Hospital of Harbin Medical University, Harbin, 150086, China.
    300 rdf:type schema:Organization
     




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