Dentin Sialophosphoprotein Knockout Mouse Teeth Display Widened Predentin Zone and Develop Defective Dentin Mineralization Similar to Human Dentinogenesis Imperfecta Type III*

  title={Dentin Sialophosphoprotein Knockout Mouse Teeth Display Widened Predentin Zone and Develop Defective Dentin Mineralization Similar to Human Dentinogenesis Imperfecta Type III*},
  author={Taduru L. Sreenath and Tamizchelvi Thyagarajan and Bradford E. Hall and Glenn Longenecker and Rena N. D'Souza and Sung Hong and J. Tim Wright and Mary Macdougall and John J. Sauk and Ashok B. Kulkarni},
  journal={Journal of Biological Chemistry},
  pages={24874 - 24880}
Dentin sialophosphoprotein (Dspp) is mainly expressed in teeth by the odontoblasts and preameloblasts. The Dspp mRNA is translated into a single protein, Dspp, and cleaved into two peptides, dentin sialoprotein and dentin phosphoprotein, that are localized within the dentin matrix. Recently, mutations in this gene were identified in human dentinogenesis imperfecta II (Online Mendelian Inheritance in Man (OMIM) accession number 125490) and in dentin dysplasia II (OMIM accession number 125420… 

Dentin sialoprotein and dentin phosphoprotein have distinct roles in dentin mineralization.

Mutant Dentin Sialophosphoprotein Causes Dentinogenesis Imperfecta

In vitro studies showed that the secretion of the mutant DSPP was impaired and accumulated within endoplasmic reticulum, and Immunohistochemistry showed that while the immunostaining signals of dentin sialoprotein were decreased in the dentin matrix, they were remarkably increased in the odontoblasts of the DsppP19L/+ and DsPP19L/P 19L mice.

Accelerated enamel mineralization in Dspp mutant mice.

Deletion of Dentin Matrix Protein-1 Leads to a Partial Failure of Maturation of Predentin into Dentin, Hypomineralization, and Expanded Cavities of Pulp and Root Canal during Postnatal Tooth Development*

It is shown that Dmp-1 null mice postnatally develop a profound tooth phenotype characterized by a partial failure of maturation ofpredentin into dentin, enlarged pulp chambers, increased width of predentin zone with reduced dentin wall, and hypomineralization, which suggests that DMP-1 is essential for later dentinogenesis during postnatal development.

Transcriptional Repression of the Dspp Gene Leads to Dentinogenesis Imperfecta Phenotype in Col1a1-Trps1 Transgenic Mice

  • D. NapieralaYao Sun Brendan H. Lee
  • Biology, Medicine
    Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
  • 2012
It is demonstrated that mice overexpressing the Trps1 transcription factor in dentin‐producing cells, odontoblasts, present with severe defects of dentin formation that resemble DGI, providing novel insights into mechanisms of transcriptional dysregulation that leads to DGI.

Dentin phosphoprotein compound mutation in dentin sialophosphoprotein causes dentinogenesis imperfecta type III

DGI‐III is allelic with some forms of DGI‐II with and without progressive hearing loss and dentin dysplasia type II that have been shown to be caused by mutations within the DSP coding or signal peptide regions.

Dentin sialophosphoprotein: a regulatory protein for dental pulp stem cell identity and fate.

Developmental abnormalities not previously reported, such as circular dentin formation within dental pulp cells and altered odontoblast differentiation in DSPP-KO mice, are found and chondrocyte-like cells in the dental pulp from KO-mice teeth are identified.

Enamel Defects Associated With Dentin Sialophosphoprotein Mutation in Mice

The results suggest that mutant P19L-DSPP protein caused developmental enamel defects in mice, which may be associated with intracellular retention of mutant DSPP in the presecretory ameloblasts.



Reduced Expression of Dentin Sialophosphoprotein Is Associated with Dysplastic Dentin in Mice Overexpressing Transforming Growth Factor-β1 in Teeth*

In vivo evidence suggesting that TGF-β1 mediated expression ofdspp is crucial for dentin mineralization is provided and direct experimental evidence indicating that decreased dspp gene expression along with the other cellular changes in odontoblasts may result in human hereditary dental disorders like dentinogenesis imperfecta II and dentin dysplasia is provided.

Refined mapping of the human dentin sialophosphoprotein (DSPP) gene within the critical dentinogenesis imperfecta type II and dentin dysplasia type II loci.

  • M. Macdougall
  • Medicine, Biology
    European journal of oral sciences
  • 1998
Both DSPP and DMP-1 have been placed on the physical map of human chromosome 4 within the interval defined by markers D 4S564 and D4S1292, and D SPP is thereby strengthened as a candidate gene for both DGI-II and DD-II.

Dentin sialoprotein, dentin phosphoprotein, enamelysin and ameloblastin: tooth-specific molecules that are distinctively expressed during murine dental differentiation.

The interrelated expression profiles found for these tooth-specific molecules illustrate the importance of a specific molecular network to initiate highly regulated processes such as cytodifferentiation and the subsequent mineralization.

Developmental expression of a 53 KD dentin sialoprotein in rat tooth organs.

Rat dentin contains a major sialic acid-rich glycoprotein, DSP, with an overall composition similar to that of bone sialoproteins but whose biological role in dentinogenesis is unknown, and its appearance during odontoblast differentiation is suggested.

Gene Expression Patterns of Murine Dentin Matrix Protein 1 (Dmp1) and Dentin Sialophosphoprotein (DSPP) Suggest Distinct Developmental Functions In Vivo

  • R. D'SouzaA. Cavender M. Macdougall
  • Biology
    Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
  • 1997
The in vivo temporospatial expression patterns of two dentin NCP genes, dentin matrix protein 1 (Dmp1), and dentin sialophosphoprotein (DSPP) in developing molars are compared, implying that these molecules serve different biological functions in vivo.

Dentin Phosphoprotein and Dentin Sialoprotein Are Cleavage Products Expressed from a Single Transcript Coded by a Gene on Human Chromosome 4

The data suggest that the previously identified dentin extracellular matrix proteins, dentin sialoprotein and dentin phosphoprotein, are expressed as a single cDNA transcript coding for a protein that is specifically cleaved into two smaller polypeptides with unique physical-chemical characteristics.

Mapping of the human dentin matrix acidic phosphoprotein gene (DMP1) to the dentinogenesis imperfecta type II critical region at chromosome 4q21.

Dentinogenesis imperfecta type II (DGI1) is an autosomal dominant disorder of dentin formation, which has been mapped to human chromosome 4q12-q21 and it is shown that it is tightly linked to DGI1 in two families (Zmax = 11.01, theta = 0.001).

Dentin matrix proteins.

  • W. Butler
  • Biology
    European journal of oral sciences
  • 1998
Dentinogenesis consists of highly controlled events occurring a short distance from the periphery of odontoblasts: it involves formation of extracellular collagen fibrils that act as an undergirding

Genomic Organization, Chromosomal Mapping, and Promoter Analysis of the Mouse Dentin Sialophosphoprotein (Dspp) Gene, Which Codes for Both Dentin Sialoprotein and Dentin Phosphoprotein*

The structural organization of the mouse dentin sialophosphoprotein gene confirms the finding that both dent in sialoprotein and dentin phosphoprotein are encoded by a single gene with a continuous open reading frame.