Property Summary

NCBI Gene PubMed Count 62
PubMed Score 679.58
PubTator Score 249.87

Knowledge Summary


No data available


  Disease (7)


  Differential Expression (6)

Disease log2 FC p
osteosarcoma -1.046 2.4e-05
atypical teratoid / rhabdoid tumor -1.200 1.7e-04
glioblastoma -1.200 4.0e-04
medulloblastoma, large-cell -1.100 6.7e-03
adult high grade glioma -1.400 9.5e-04
ovarian cancer -1.600 2.3e-06

Gene RIF (47)

26829730 A novel pathogenic splicing-mutation c.52-1G>A of DSPP is associated with dentinogenesis imperfecta shields type II.
25805840 expression of MMP-20 and co-expression and potential interaction with DSPP in human major salivary gland tissues
25396425 Adhesive and migratory effects of phosphophoryn are modulated by flanking peptides of the integrin binding motif.
25158199 mutations of the DSP-PP P4 to P4' cleavage site can block, impair or accelerate dentin sialoprotein phosphophoryn cleavage, and suggest that its Bone morphogenic protein 1 cleavage site is conserved in order to regulate its cleavage efficiency
24441185 DMP1 and DSPP were more abundant in carious than in sound samples.
24400037 Domain of dentine sialoprotein mediates proliferation and differentiation of human periodontal ligament stem cells.
23589294 DSS domain of DPP functions as a novel cell-penetrating peptide, and these findings demonstrate new opportunities for intracellular delivery of therapeutic proteins and cell tracking in vivo.
23509818 analysis of a mutation in DSPP causing dentinogenesis imperfecta and characterization of the mutational effect
23297400 efficiency of dentin sialoprotein-phosphophoryn processing is affected by mutations both flanking and distant from the cleavage site
22521702 A review of hereditary dentine diseases resulting from mutations in DSPP gene suggests that the localization of mutation in the sequence of the DSPP gene might result in a different phenotype due to the diverse cellular fate of the mutated protein.
22410369 DSPP, OPN, or MMP-9 expressions at histologically-negative surgical margins predict Oral squamous cell carcinoma recurrence with MMP-9 being the preferred predictor.
22392858 This study presents evidence of a shared underlying mechanism of capturing of normal DSPP by two different classes of DSPP mutations.
22392858 Data shows all known DSPP mutations (except Y6D) cause nonsyndromic dentin dysplasia,DD-II, and dentinogenesis imperfecta, DGI II & III, by retention of mutant proteins in the endoplasmic reticulum with associated decreased secretion of normal DSPP.
22310900 The P17 residue of DSPP is a mutational hotspot in a Chinese family with Dentinogenesis Imperfecta type II.
22243242 study concludes that enamel defects can be part of the dental phenotype caused by DSPP mutations, although DSPP is not critical for dental enamel formation
22125647 Data show that the novel dentin sialophosphoprotein (DSPP) mutation was considered as the causation of dentinogenesis imperfecta type II (DGI-II).
21736673 predictions of exon 3 skipping in specific DSPP mutations have been validated; cryptic splicing donor site has been identified. possible insight into DSPP mutations in the pathogenesis and genotype-phenotype correlations of hereditary dentin defects.
21687927 Results suggest that DSPP is regulated post-transcriptionally by mir32, mir885-5p and mir586 during odontoblast differentiation.
21103065 Data show there was a direct correlation between the degree of DSPP-silencing and suppression of MMP-2, MMP-3 and MMP-9.
21029264 DSPP gene mutation not only influences dentinogenesis but also affects early stage amelogenesis.
20949630 Frameshift mutations in the part of the DSPP gene coding for DPP explain a significant part of inherited and isolated dentin diseases, i.e., dentin dysplasia type II and dentinogenesis imperfecta variants.
20802180 High dentin expression is associated with dental caries.
20618350 Data identified novel single bp deletional DSPP mutations in three Korean families with DGI type II.
20367116 This review of genetic studies demonstrates that mutations in, or knockout of the Dspp gene result in mineralization defects in dentin and/or bone.
20201926 Observational study and genome-wide association study of gene-disease association. (HuGE Navigator)
20146806 Direct DNA sequencing identified a novel A-->G transition mutation adjacent to the donor splicing site within intron 3 in all affected individuals.
20121932 Mutation analysis revealed mutation (c.53T>A, p.V18D, g.1192T>A) involving 2nd nucleotide of 1st codon within exon 3 of DSPP gene. This is 7th mutation in DSPP V18 residue. Only 1 other was shown as de novo mutation; it also affected V18 AA residue.
19834535 Observational study of gene-disease association. (HuGE Navigator)
19806576 A novel mutation in the first exon of the DSPP gene were found in all dentinogenesis imperfecta patients in a Chinese family.
19131317 A severely affected primary dentition with wide-open pulp chambers and multiple pulp exposures resembling a DGI-III pattern was analyzed to reveal a novel mutation (c.53T>A, p.V18D) near the intron-exon boundary in the third exon of the DSPP gene.
19103209 The heterozygous deletion mutation in DSPP contributed to the pathogenesis of dentinogenesis imperfecta type II.
19029076 A novel -1 bp frameshift (c.3141delC) falls within the portion of the DSPP repeat domain previously associated solely with the DGI phenotype. This new frameshift mutation shows that overlapping DSPP mutations can give rise to either DGI or DD phenotypes.
19026876 Identification of DSPP splice junction mutation (IVS2-6T>G) in a family with dentin dysplasia type II. Mutation is in 6th nucleotide from the end of intron 2, perfectly segregates with the disease phenotype.
18797159 The aim of this study was to perform phenotype analysis and dentin sialophosphoprotein (DSPP) mutational analysis on 3 Brazilian families diagnosed with dentinogenesis imperfecta type II.
18521831 Within 9 dentin dysplasia (type II) and dentinogenesis imperfecta (type II and III) patient/families, 7 have 1 of 4 net -1 deletions within the a 2-kb coding repeat domain of the DSPP gene while the remaining 2 patients have splice-site mutations.
18456718 Data provide the first evidence that DPP mutations can cause hereditary dentin disorders and suggest that in-frame length variations and missense SNPs in DPP have no obvious pathogenetic effects on dentin formation.
18211748 The results show high expression levels of DSPP in human tooth germs indicating that it may play an essential role for physiological and pathological events in tooth development.
17686168 mutation p.Pro17Ser causes type II dentinogenesis imperfecta in the Chinese family
16920545 2 mutation hotspots may be causative for multiple unrelated dentinogenesis imperfecta families with different clinical phenotypes
16679514 DMP1 regulates the expression of the DSPP gene
16567553 Mutational analyses identified no coding or intron junction sequence variations associated with affection status in DMP1, MEPE, or the DSP portion of DSPP. The defects in the permanent dentition were typically mild and consistent with DD-II.
16108038 study shows for the first time that DSPP is ectopically expressed in human prostate cancer; expression of this SIBLING protein strongly correlates with conventional histopathological prognostic indicators of prostate cancer progression
16014627 Dentin sialophosphoprotein expressed by transgenic presecretory ameloblasts contributes to the unique properties of the dentino-enamel junction.
15954904 DSPP is expressed in the proximal and distal tubules of kidney nephron.
15371433 there is a novel signaling function for phosphophoryn in cell differentiation beyond the hypothesized role of PP in biomineralization
15329369 DSPP is expressed in salivary gland ducts, particularly the striated duct.
11856645 Transient DSPP expression was seen in the presecretory ameloblasts with continuous expression in the odontoblasts.

AA Sequence

STSDSNDESDSQSKSGNGNNNGSDSDSDSEGSDSNHSTSDD                                1261 - 1301

Text Mined References (66)

PMID Year Title
26829730 2016 [Identification of a novel mutation of DSPP gene in a Chinese family affected with dentinogenesis imperfecta shields type II].
25805840 2015 Expression of Matrix Metalloproteinase (MMP)-20 and Potential Interaction with Dentin Sialophosphoprotein (DSPP) in Human Major Salivary Glands.
25396425 2014 Adhesive and migratory effects of phosphophoryn are modulated by flanking peptides of the integrin binding motif.
25158199 2014 Site specificity of DSP-PP cleavage by BMP1.
24441185 2013 Dentin matrix protein 1 and dentin sialophosphoprotein in human sound and carious teeth: an immunohistochemical and colorimetric assay.
24400037 2013 Domain of dentine sialoprotein mediates proliferation and differentiation of human periodontal ligament stem cells.
23589294 2013 Acidic domain in dentin phosphophoryn facilitates cellular uptake: implications in targeted protein delivery.
23509818 2013 A DSPP mutation causing dentinogenesis imperfecta and characterization of the mutational effect.
23297400 2013 The efficiency of dentin sialoprotein-phosphophoryn processing is affected by mutations both flanking and distant from the cleavage site.
23064961 2013 GWAS of dental caries patterns in the permanent dentition.
22534175 2012 Dentin sialophosphoprotein and dentin matrix protein-1: Two highly phosphorylated proteins in mineralized tissues.
22521702 2012 Hereditary dentine diseases resulting from mutations in DSPP gene.
22410369 2012 Expressions of matrix metalloproteinase-9 (MMP-9), dentin sialophosphoprotein (DSPP), and osteopontin (OPN) at histologically negative surgical margins may predict recurrence of oral squamous cell carcinoma.
22392858 2012 Rough endoplasmic reticulum trafficking errors by different classes of mutant dentin sialophosphoprotein (DSPP) cause dominant negative effects in both dentinogenesis imperfecta and dentin dysplasia by entrapping normal DSPP.
22310900 2012 Mutation identification of the DSPP in a Chinese family with DGI-II and an up-to-date bioinformatic analysis.
22243242 2011 Enamel malformations associated with a defined dentin sialophosphoprotein mutation in two families.
22125647 2011 A novel splicing mutation alters DSPP transcription and leads to dentinogenesis imperfecta type II.
21736673 2011 Functional splicing assay of DSPP mutations in hereditary dentin defects.
21687927 2011 miRNA expression profiling identifies DSPP regulators in cultured dental pulp cells.
21103065 2010 Dentin sialophosphoprotein (DSPP) gene-silencing inhibits key tumorigenic activities in human oral cancer cell line, OSC2.
21029264 2011 Identification of the DSPP mutation in a new kindred and phenotype-genotype correlation.
20949630 2011 Frameshift mutations in dentin phosphoprotein and dependence of dentin disease phenotype on mutation location.
20802180 2010 Adaptive calcified matrix response of dental pulp to bacterial invasion is associated with establishment of a network of glial fibrillary acidic protein+/glutamine synthetase+ cells.
20618350 2011 Novel dentin phosphoprotein frameshift mutations in dentinogenesis imperfecta type II.
20367116 2010 Dentin sialophosphoprotein in biomineralization.
20201926 2010 Human variation in alcohol response is influenced by variation in neuronal signaling genes.
20146806 2010 A novel DSPP mutation causes dentinogenesis imperfecta type II in a large Mongolian family.
20121932 2009 De novo mutation in the DSPP gene associated with dentinogenesis imperfecta type II in a Japanese family.
20079836 2010 Dentin sialophosphoprotein (DSPP) is cleaved into its two natural dentin matrix products by three isoforms of bone morphogenetic protein-1 (BMP1).
19834535 2009 Sequential use of transcriptional profiling, expression quantitative trait mapping, and gene association implicates MMP20 in human kidney aging.
19806576 2009 [Mutation analysis of a Chinese family with genetic dentinogenesis imperfecta].
19131317 2009 A novel mutation in the DSPP gene associated with dentinogenesis imperfecta type II.
19103209 2009 A novel splice site mutation in the dentin sialophosphoprotein gene in a Chinese family with dentinogenesis imperfecta type II.
19029076 2008 Overlapping DSPP mutations cause dentin dysplasia and dentinogenesis imperfecta.
19026876 2008 A dentin sialophosphoprotein mutation that partially disrupts a splice acceptor site causes type II dentin dysplasia.
18797159 2009 Phenotype characterization and DSPP mutational analysis of three Brazilian dentinogenesis imperfecta type II families.
18521831 2008 A comprehensive analysis of normal variation and disease-causing mutations in the human DSPP gene.
18456718 2008 Dentin phosphoprotein frameshift mutations in hereditary dentin disorders and their variation patterns in normal human population.
18211748 2007 Dentin sialophosphoprotein expression during human matrix development.
17686168 2007 A novel DSPP mutation is associated with type II dentinogenesis imperfecta in a Chinese family.
17627120 2007 Disorders of human dentin.
17033625 2006 DMP1 mutations in autosomal recessive hypophosphatemia implicate a bone matrix protein in the regulation of phosphate homeostasis.
16920545 2006 Phenotypes and genotypes in 2 DGI families with different DSPP mutations.
16679514 2006 Dentin matrix protein 1 regulates dentin sialophosphoprotein gene transcription during early odontoblast differentiation.
16567553 2006 Phenotypic variation in dentinogenesis imperfecta/dentin dysplasia linked to 4q21.
16108038 2006 Expression of dentin sialophosphoprotein in human prostate cancer and its correlation with tumor aggressiveness.
16014627 2005 Dentin sialoprotein and dentin phosphoprotein overexpression during amelogenesis.
15954904 2005 Renal expression of SIBLING proteins and their partner matrix metalloproteinases (MMPs).
15815621 2005 Generation and annotation of the DNA sequences of human chromosomes 2 and 4.
15592686 2005 Mutational hot spot in the DSPP gene causing dentinogenesis imperfecta type II.
15371433 2004 Phosphophoryn regulates the gene expression and differentiation of NIH3T3, MC3T3-E1, and human mesenchymal stem cells via the integrin/MAPK signaling pathway.
15329369 2004 Expression of SIBLINGs and their partner MMPs in salivary glands.
14758537 2004 Clinical, histopathologic, and genetic investigation in two large families with dentinogenesis imperfecta type II.
12354781 2002 Mutation of the signal peptide region of the bicistronic gene DSPP affects translocation to the endoplasmic reticulum and results in defective dentine biomineralization.
12097430 2002 The expression of dentin sialophosphoprotein gene in bone.
11856645 2002 Investigation of osteocalcin, osteonectin, and dentin sialophosphoprotein in developing human teeth.
11175790 2001 Dentinogenesis imperfecta 1 with or without progressive hearing loss is associated with distinct mutations in DSPP.
11175779 2001 DSPP mutation in dentinogenesis imperfecta Shields type II.
10980418 2000 A substractive PCR-based cDNA library from human odontoblast cells: identification of novel genes expressed in tooth forming cells.
10706475 2000 Molecular cloning of a human dentin sialophosphoprotein gene.
9879917 1998 Human dentin phosphophoryn nucleotide and amino acid sequence.
9541230 1998 Refined mapping of the human dentin sialophosphoprotein (DSPP) gene within the critical dentinogenesis imperfecta type II and dentin dysplasia type II loci.
9541227 1998 Dentin matrix proteins.
9533027 1997 Assignment of dentin sialophosphoprotein (DSPP) to the critical DGI2 locus on human chromosome 4 band q21.3 by in situ hybridization.
9493074 Dentin dysplasia, type II linkage to chromosome 4q.
8995371 1997 Dentin phosphoprotein and dentin sialoprotein are cleavage products expressed from a single transcript coded by a gene on human chromosome 4. Dentin phosphoprotein DNA sequence determination.