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Rabbit Anti-FAM20C/BF488 Conjugated antibody (bs-12376R-BF488)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-12376R-BF488
英文名稱 Rabbit Anti-FAM20C/BF488 Conjugated antibody
中文名稱 BF488標記的胞外分泌型絲氨酸/蘇氨酸蛋白激酶FAM20C抗體
別    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.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  發(fā)育生物學  信號轉導  干細胞  激酶和磷酸酶  細胞外基質  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Rat,  (predicted: Human, Mouse, Chicken, Dog, Cow, Horse, Sheep, Monkey, )
產品應用 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    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FAM20C
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 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.
產品介紹 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.
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