FITC标记的狄乔治(DiGeorge)氏综合征相关蛋白抗体-抗体-抗体-生物在线
FITC标记的狄乔治(DiGeorge)氏综合征相关蛋白抗体

FITC标记的狄乔治(DiGeorge)氏综合征相关蛋白抗体

商家询价

产品名称: FITC标记的狄乔治(DiGeorge)氏综合征相关蛋白抗体

英文名称: Anti-DGCR14/FITC

产品编号: HZ-14292R-FITC

产品价格: null

产品产地: 中国/上海

品牌商标: HZbscience

更新时间: 2023-08-17T10:24:20

使用范围: ICC=1:50-200 IF=1:50-200

上海沪震实业有限公司
  • 联系人 : 鲍丽雯
  • 地址 : 上海市闵行区闵北路88弄1-30号第22幢AQ136室
  • 邮编 : 200612
  • 所在区域 : 上海
  • 电话 : 139****0749
  • 传真 : 021-60345367
  • 邮箱 : www.shzbio.net

 Rabbit Anti-DGCR14/FITC Conjugated antibody

FITC标记的狄乔治(DiGeorge)氏综合征相关蛋白抗体

 

英文名称 Anti-DGCR14/FITC
中文名称 FITC标记的狄乔治(DiGeorge)氏综合征相关蛋白抗体
别    名 DGCR14; DGS H; DGS I; DGSH; DGSI; DiGeorge syndrome critical region gene 14; DiGeorge syndrome critical region gene DGSI; DiGeorge syndrome critical region protein 14; DiGeorge syndrome gene H; DiGeorge syndrome gene I; ES2; DGC14_HUMAN; ES2 protein; Es2el.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 心血管  发育生物学  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Rabbit, 
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 53kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DGCR14
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:
DGCR14 is a 476 amino acid nuclear protein that belongs to the DGCR14 family. DGCR14 is believed to play a part in the etiology of the velocardiofacial/DiGeorge syndrome (VCFS/DGS), a developmental disorder characterized by structural and functional palate anomalies, conotruncal cardiac malformations, immunodeficiency, hypocalcemia, and typical facial anomalies. Most cases result from a deletion of chromosome 22q11.2 (DiGeorge syndrome chromosome region, or DGCR). This protein localizes to the nucleus and co-purifies with C complex spliceosomes.

Function:
DiGeorge syndrome critical region 14, DGCR14 has been identified in the spliceosome C complex and may be involved in pre-mRNA splicing. May have a role in velocardiofacial/DiGeorge syndrome (VCFS/DGS), a developmental disorder characterized by structural and functional palate anomalies, conotruncal cardiac malformations, immunodeficiency, hypocalcemia, and facial anomalies. Most cases of this syndrome involve a deletion of chromosome 22q11.2 in the DiGeorge syndrome chromosome region. 

Subcellular Location:
Nuclear

Similarity:
Belongs to the DGCR14 family.

Database links:

Entrez Gene: 8220 Human

Omim: 601755 Human

SwissProt: Q96DF8 Human

Unigene: 517407 Human

Unigene: 686112 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic application
   

 

DGCR14是属于DGCR14家族的476个氨基酸的核蛋白。DGCR14被认为在心速/DiGeorge综合征(VCFS/DGS)的病因学上起作用,VCFS/DGS是一种发育性疾病,特征是结构和功能腭部异常、心梗畸形、免疫缺陷、低钙血症和典型的面部异常。大多数病例是由染色体22q11.2缺失(DigeRoGE综合征染色体区,或DGCR)引起的。该蛋白定位于细胞核,并与C复合剪接体共同纯化。