牙本质骨唾液酸糖蛋白/牙本质骨唾液酸磷蛋白抗体

英文名称    DSPP    

中文名称    牙本质骨唾液酸糖蛋白/牙本质骨唾液酸磷蛋白抗体    

别    名    Dentin phosphophoryn; Dentin phosphoprotein; dentin phosphoryn; Dentin sialophosphoprotein; Dentin sialophosphoprotein precursor; Dentin sialoprotein; dentinogenesis imperfecta 1; DFNA39; DGI1; DMP3; DPP; DSP; Dspp; DSPP_HUMAN; DTDP2.    

说 明 书    0.1ml  0.2ml      

研究领域    细胞生物  发育生物学  信号转导  细胞周期蛋白  结合蛋白  细胞分化  细胞骨架  细胞外基质      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat,     

产品应用    IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 牙本质骨唾液酸糖蛋白/牙本质骨唾液酸磷蛋白抗体（石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.    

分 子 量    47/129kDa    

细胞定位    细胞外基质 分泌型蛋白     

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human Dentin sialoprotein    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4    

保存条件    Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.    

牙本质骨唾液酸糖蛋白/牙本质骨唾液酸磷蛋白抗体产品介绍    background:

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]

Function:
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.

Subunit:
Interacts with FBLN7.

Subcellular Location:
Secreted, extracellular space, extracellular matrix. 

Tissue Specificity:
Expressed in teeth. DPP is synthesized by odontoblast and transiently expressed by pre-ameloblasts.

Post-translational modifications:
DSP is glycosylated. 

DISEASE:
Defects in DSPP are the cause of deafness autosomal dominant type 39 with dentinogenesis imperfecta 1 (DFNA39/DGI1) [MIM:605594]. 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) [MIM:125490]; 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) [MIM:125500]. 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) [MIM:125420]; 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.     

牙本质骨唾液酸糖蛋白/牙本质骨唾液酸磷蛋白抗体

hz-2888R TRPM2  瞬时受体电位离子通道蛋白/M亚家族抗体

hz-2804R Troponin T/cTnT  心肌特异性肌钙蛋白T抗体

hz-1217R TSARG3/TSARG1  生精细胞凋亡相关基因3抗体

hz-1220R TSARG4  生精细胞凋亡相关基因4抗体

hz-1923R TSARG6/DNAJB13  生精细胞凋亡相关基因6抗体

hz-1924R TSARG7  精子形成相关凋亡蛋白7抗体

hz-0716R CIDEB  CIDEB抗体

hz-1365R Tsg101  肿瘤易感基因101蛋白抗体

hz-2676R TSH  促甲状腺素抗体

hzm-2676M TSHB(2T11)  促甲状腺素单克隆抗体

hz-2676M TSHB  小鼠抗促甲状腺素抗体

hz-0460R TSHR  促甲状腺素受体抗体

hz-0206R TSHR (CT)  促甲状腺素受体抗体（C端）

hz-0003R TSHR (NT)  促甲状腺素受体抗体（N端）

hz-2087R TSHR  促甲状腺素受体抗体

hz-1147R SynCAM/TSLC1  肺癌肿瘤阻抑基因1抗体

hz-0449R TSLP (human)  胸腺基质淋巴细胞生成素-1抗体(人)

hz-2727R CRLF2  胸腺基质淋巴细胞生成素受体抗体

hz-2715R TSP-1/THBS1  血小板反应蛋白抗体

hz-0826R TTF-1  甲状腺核转录因子-1抗体

hz-0446R TTF-2  甲状腺转录因子-2抗体

hz-0159R Tubulin-alpha  微管蛋白α抗体 Tubulin α

hz-5118R Tubulin alpha 6/TUBA1C  微管相关蛋白α6抗体

hz-0210R Tubulin-beta  微管蛋白抗体（免疫组化用抗体）Tubulinβ

hz-0715R Tubulin-Beta  微管蛋白抗体（免疫组化用抗体）Tubulin β

hz-4511R tubulin Beta  微管蛋白β tubulin抗体 Tubulin β

hz-4512R TUBB3/beta III Tubulin(Neuronal Marker)  神经细胞特异性微管蛋白抗体

hz-5607R phospho-TUBB3(Ser172)  磷酸化神经细胞特异性微管蛋白抗体

hz-1322R gamma tubulin(Centrosome Marker )  微管蛋白-γ抗体

hz-2160R Tumstatin/COL4A3  肿瘤抑素抗体

hz-2670R Tubb3/Tubulin beta 3  微管蛋白β3抗体

hz-2441R TWIST protein  TWIST蛋白抗体