NP_055023.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
131,151 Da
NCBI Official Full Name
dentin sialophosphoprotein preproprotein
NCBI Official Synonym Full Names
dentin sialophosphoprotein
NCBI Official Synonym Symbols
DPP; DSP; DGI1; DMP3; DTDP2; DFNA39 [Similar Products]
NCBI Protein Information
dentin sialophosphoprotein; dentin phosphoryn; dentin sialoprotein; dentin phosphophoryn; dentin phosphoprotein
UniProt Protein Name
Dentin sialophosphoprotein
UniProt Synonym Protein Names
Dentin phosphophoryn
UniProt Synonym Gene Names
UniProt Entry Name
DSPP_HUMAN
NCBI Summary for DSPP
This gene encodes two principal proteins of the dentin extracellular matrix of the tooth. The preproprotein is secreted by odontoblasts and cleaved into dentin sialoprotein and dentin phosphoprotein. Dentin phosphoprotein is thought to be involved in the biomineralization process of dentin. Mutations in this gene have been associated with dentinogenesis imperfecta-1; in some individuals, dentinogenesis imperfecta occurs in combination with an autosomal dominant form of deafness. Allelic differences due to repeat polymorphisms have been found for this gene. [provided by RefSeq, Jul 2008]
UniProt Comments for DSPP
DSPP: DSP may be an important factor in dentinogenesis. DPP may bind high amount of calcium and facilitate initial mineralization of dentin matrix collagen as well as regulate the size and shape of the crystals. Defects in DSPP are the cause of deafness autosomal dominant type 39 with dentinogenesis imperfecta 1 (DFNA39/DGI1). Affected individuals present DGI1 associated with early onset progressive sensorineural high-frequency hearing loss. Defects in DSPP are the cause of dentinogenesis imperfecta type 1 (DGI1); also known as dentinogenesis imperfecta Shields type 2 (DGI2). DGI1 is an autosomal dominant disorder in which both the primary and the permanent teeth are affected. It occurs with an incidence of 1:8000 live births. The teeth are amber and opalescent, the pulp chamber being obliterated by abnormal dentin. The enamel, although unaffected, tends to fracture, which makes dentin undergo rapid attrition, leading to shortening of the teeth. Defects in DSPP are a cause of dentinogenesis imperfecta Shields type 3 (DGI3). Patients with DGI3 do not have stigmata of osteogenesis imperfecta. The finding that a single defects in the DSPP gene causes both phenotypic patterns of DGI2 and DGI3 strongly supports the conclusion that these two disorders are not separate diseases but rather the phenotypic variation of a single genetic defect. Defects in DSPP are the cause of dentin dysplasia type 2 (DTDP2); also known as dentin dysplasia Shields type 2. DTDP2 is an autosomal dominant disorder in which mineralization of the dentine of the primary teeth is abnormal. On the basis of the phenotypic overlap between, and shared chromosomal location with DGI2 it has been proposed that DTDP2 and DGI2 are allelic. From the results of recent studies, it is clear that different types of mutations in DSPP lead to the two different phenotypes.
Protein type: Secreted; Secreted, signal peptide; Extracellular matrix
Chromosomal Location of Human Ortholog: 4q21.3
Cellular Component: proteinaceous extracellular matrix; cytoplasm; extracellular region
Molecular Function: collagen binding; extracellular matrix structural constituent; calcium ion binding
Biological Process: extracellular matrix organization and biogenesis; ossification; biomineral formation; multicellular organismal development; skeletal development
Disease: Dentinogenesis Imperfecta, Shields Type Iii; Deafness, Autosomal Dominant 39, With Dentinogenesis Imperfecta 1; Dentin Dysplasia, Type Ii; Dentinogenesis Imperfecta 1
Research Articles on DSPP
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Diseases associated with DSPP elisa kit
Organs/Tissues associated with DSPP elisa kit
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