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货号： bs-12376R-PE-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-12376R-PE-Cy7
- 英文名称
- Anti-FAM20C/PE-Cy7
- 中文名称
- PE-Cy7标记的胞外分泌型丝氨酸/苏氨酸蛋白激酶FAM20C抗体
- 别 名
- C76981; Dentin matrix protein 4; DKFZp547C074; DMP-4; DMP4; DMP4_HUMAN; Extracellular serine/threonine protein kinase Fam20C; Fam20c; Family with sequence similarity 20 member C; GEF CK; Golgi enriched fraction casein kinase; Protein FAM20C; RNS antibod.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 发育生物学 信号转导 干细胞 激酶和磷酸酶 细胞外基质
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Sheep, Monkey,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 64kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human FAM20C
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 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.
- 产品介绍
- background:
The FAM20 proteins are a family of secreted proteins that regulate differentiation and function of hematopoietic and other tissues. FAM20C, also known as DMP4 (Dentin matrix protein 4), is a 570 amino acid secreted protein that binds calcium and may play a role in dentin mineralization. Defects in the gene encoding FAM20C are the cause of Raine syndrome (Lethal osteosclerotic bone dysplasia), an autosomal recessive osteosclerotic bone dysplasia, that is characterized by generalized osteosclerosis, microencephaly and craniofacial dysplasia. Usually, affected individuals survive only days or weeks. The mutations of the FAM20C gene include four nonsynomous base changes and four splice-site changes that have a detrimental affect on splicing.
Function:
Calcium-binding protein which may play a role in dentin mineralization.
Subcellular Location:
Secreted.
Tissue Specificity:
Widely expressed.
DISEASE:
Defects in FAM20C are the cause of Raine syndrome (RNS) [MIM:259775]. RNS is an autosomal recessive osteosclerotic bone dysplasia with neonatal lethal outcome. Clinical features include generalized osteosclerosis, craniofacial dysplasia and microcephaly.
Similarity:
Belongs to the FAM20 family.
Database links:
UniProtKB/Swiss-Prot: Q8IXL6.2
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.