antibody

DSPP Polyclonal Antibody

MBS2541229

0.06 mL

150 USD

polyclonal

rabbit

nonconjugated

Antibody

DSPP Polyclonal Antibody

[DSPP]

[DPP; DSP; DGI1; DMP3; DTDP2; DFNA39]

[dentin sialophosphoprotein preproprotein; Dentin sialophosphoprotein; dentin sialophosphoprotein; dentin sialophosphoprotein; Dentin phosphoproteinAlternative name(s):Dentin phosphophoryn; DPP]

[DSPP]

[DSPP; DSPP; DPP; DSP; DGI1; DMP3; DFNA39; DPP; DSP]

DSPP_HUMAN

Polyclonal

IgG

Rabbit

Human, Mouse, Rat

1301

Affinity Purification

Store at -20 degree C. Avoid freeze/thaw cycles.

Western Blot (WB)

WB: 1:500 - 1:2000

Western Blot (WB)

Immunogen: Recombinant protein of human DSPP.

Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. Calculated MW: 131kDa . Observed MW: 110kDa

89001107

NM_014208.3

ECM Proteoglycans Pathway (1270256); Extracellular Matrix Organization Pathway (1270244)

This gene 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. This gene is present in a gene cluster on chromosome 4. Allelic differences due to repeat polymorphisms have been found for this gene. [provided by RefSeq, Jan 2016]

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: Extracellular matrix; Secreted; Secreted, signal peptide. Chromosomal Location of Human Ortholog: 4q21.3. Cellular Component: cytoplasm; extracellular region. Molecular Function: calcium ion binding; collagen binding; extracellular matrix structural constituent. Biological Process: extracellular matrix organization and biogenesis; multicellular organismal development; ossification; skeletal development. Disease: Deafness, Autosomal Dominant 39, With Dentinogenesis Imperfecta 1; Dentin Dysplasia, Type Ii; Dentinogenesis Imperfecta 1; Dentinogenesis Imperfecta, Shields Type Iii

0.06 mL

150 USD

0.12 mL

225

0.2 mL

360