Bioinformatics Database
DSPP: Dentin sialophosphoprotein
Cellular Process
Enamel and Dentin formation
Gene Name
DSPP: Dentin sialophosphoprotein
Gene ID
1834
Gene Sequence
General Description
DSPP encodes a member of the small integrin-binding ligand N-linked glycoprotein (SIBLING) family of proteins.
Alternative titles; symbols
Chromosome
Chromosome 4
Cytogenetic location
4q22.1
Encoded Protein
Dentin sialophosphoprotein preproprotein
Function of the protein in oral and tooth development
DSPP encodes a member of the small integrin-binding ligand N-linked glycoprotein (SIBLING) family of proteins. The encoded preproprotein is secreted by odontoblasts and proteolytically processed to generate two principal proteins of the dentin extracellular matrix of the tooth, dentin sialoprotein and dentin phosphoprotein. These two protein products may play distinct but related roles in dentin mineralization. Mutations in this gene are associated with dentinogenesis imperfecta and dentin dysplasia.
Dental and Oral Diseases
Deafness, autosomal dominant 39, with dentinogenesis
Dentin dysplasia, type II
Dentinogenesis imperfecta, Shields type II
Dentinogenesis imperfecta, Shields type III
(OMIM ID: 125485)
Protein Sequence
>NP_055023.2 DSPP [organism=Homo sapiens] [GeneID=1834]
MKIITYFCIWAVAWAIPVPQSKPLERHVEKSMNLHLLARSNVSVQDELNASGTIKESGVLVHEGDRGRQE
NTQDGHKGEGNGSKWAEVGGKSFSTYSTLANEEGNIEGWNGDTGKAETYGHDGIHGKEENITANGIQGQV
SIIDNAGATNRSNTNGNTDKNTQNGDVGDAGHNEDVAVVQEDGPQVAGSNNSTDNEDEIIENSCRNEGNT
SEITPQINSKRNGTKEAEVTPGTGEDAGLDNSDGSPSGNGADEDEDEGSGDDEDEEAGNGKDSSNNSKGQ
EGQDHGKEDDHDSSIGQNSDSKEYYDPEGKEDPHNEVDGDKTSKSEENSAGIPEDNGSQRIEDTQKLNHR
ESKRVENRITKESETHAVGKSQDKGIEIKGPSSGNRNITKEVGKGNEGKEDKGQHGMILGKGNVKTQGEV
VNIEGPGQKSEPGNKVGHSNTGSDSNSDGYDSYDFDDKSMQGDDPNSSDESNGNDDANSESDNNSSSRGD
ASYNSDESKDNGNGSDSKGAEDDDSDSTSDTNNSDSNGNGNNGNDDNDKSDSGKGKSDSSDSDSSDSSNS
SDSSDSSDSDSSDSNSSSDSDSSDSDSSDSSDSDSSDSSNSSDSSDSSDSSDSSDSSDSSDSKSDSSKSE
SDSSDSDSKSDSSDSNSSDSSDNSDSSDSSNSSNSSDSSDSSDSSDSSSSSDSSNSSDSSDSSDSSNSSE
SSDSSDSSDSDSSDSSDSSNSNSSDSDSSNSSDSSDSSNSSDSSDSSDSSNSSDSSDSSDSSNSSDSSDS
SDSSDSSDSSNSSDSNDSSNSSDSSDSSNSSDSSNSSDSSDSSDSSDSDSSNSSDSSNSSDSSDSSNSSD
SSDSSDSSDGSDSDSSNRSDSSNSSDSSDSSDSSNSSDSSDSSDSNESSNSSDSSDSSNSSDSDSSDSSN
SSDSSDSSNSSDSSESSNSSDNSNSSDSSNSSDSSDSSDSSNSSDSSNSSDSSNSSDSSDSNSSDSSDSS
NSSDSSDSSDSSDSSDSSDSSNSSDSSDSSDSSDSSNSSDSSNSSDSSNSSDSSDSSDSSDSSDSSDSSD
SSDSSNSSDSSDSSDSSDSSDSSDSSDSSDSSESSDSSDSSNSSDSSDSSDSSDSSDSSDSSDSSDSSDS
SNSSDSSDSSDSSDSSDSSNSSDSSDSSESSDSSDSSDSSDSSDSSDSSDSSDSSDSSNSSDSSDSSDSS
DSSDSSDSSDSSDSSDSSDSSDSSDSSDSSDSSDSSDSSDSNESSDSSDSSDSSDSSNSSDSSDSSDSSD
STSDSNDESDSQSKSGNGNNNGSDSDSDSEGSDSNHSTSDD
Mutations
Deafness, autosomal dominant 39, with dentinogenesis: PRO17THR; VAL18PHE
Dentin dysplasia, type II: ASP6TYR
Dentinogenesis imperfecta, Shields type II: GLN45TER, G-to-A transition in the donor splice site (GT) of intron 3; ARG68TRP; ALA15VAL; 1-bp deletion (2040delC); 1-bp deletion (3438delC)
(Song et al., 2008;
Related Literature
Song et al.,(2008): https://doi.org/10.1136/jmg.2007.056911
Malmgren et al., (2004): https://doi.org/10.1007/s00439-004-1084-z
Rajpar et al., (2002): https://doi.org/10.1093/hmg/11.21.2559