FITC标记的高嗜酸性粒细胞综合症相关蛋白FIP1L1抗体-抗体-抗体-生物在线
上海沪震实业有限公司
FITC标记的高嗜酸性粒细胞综合症相关蛋白FIP1L1抗体

FITC标记的高嗜酸性粒细胞综合症相关蛋白FIP1L1抗体

商家询价

产品名称： FITC标记的高嗜酸性粒细胞综合症相关蛋白FIP1L1抗体

英文名称： Anti-FIP1L1/FITC

产品编号： HZ-13173R-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

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 Rabbit Anti-FIP1L1/FITC Conjugated antibody

 

FITC标记的高嗜酸性粒细胞综合症相关蛋白FIP1L1抗体
英文名称 Anti-FIP1L1/FITC
中文名称 FITC标记的高嗜酸性粒细胞综合症相关蛋白FIP1L1抗体
别    名 DKFZp586K0717; Factor interacting with PAP; FIP1; FIP1 like 1 (S cerevisiae); FIP1 like 1; FLJ33619; hFip 1; hFip1; Pre mRNA 3 end processing factor FIP1; Rearranged in hypereosinophilia; RHE; FIP1_HUMAN.  
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  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, 
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 66kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FIP1L1
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 Component of the Cleavage and Polyadenylation Specificity Factor (CPSF) complex plays an important role in the 3'-end formation of pre-mRNA. This complex recognizes the AAUAAA signal sequence and interacts with poly(A) polymerase to process and add to the poly(A) tail. FIP1L1 (FIP1-like 1), also known as Pre-mRNA 3'-end-processing factor FIP1, FIP1 (Factor interacting with PAP) and RHE (Rearranged in hypereosinophilia), is a 594 amino acid nuclear protein that is a component of the CPSF complex. Within the complex, FIP1L1 contributes to the poly(A) recognition and stimulates poly(A) addition. Fusion of the genes encoding FIP1L1 and PDGFRA due to an interstitial deletion on chromosome 4q12 is the cause of hypereosinophilia syndrome, a rare blood disorder characterized by continuous overproduction of eosinophils in the bone marrow that leads to tissue infiltration and organ damage. There are three isoforms of FIP1L1 that are produced as a result of alternative splicing events.

Function:
FIP1L1 is an integral subunit of the cleavage and polyadenylation specificity factor (CPSF) complex. CPSF is a multisubunit complex that binds to the canonical AAUAAA hexamer and to U-rich upstream sequence elements on the pre-mRNA and interacts with poly(A) polymerase and other factors to bring about cleavage and poly(A) addition. FIP1L1 binds to U-rich RNA sequence elements surrounding the poly(A) site and contributes to poly(A) site recognition and stimulates poly(A) addition. FIP1L1 may act to tether poly(A) polymerase to the CPSF complex. A fusion of FIP1L1 and PDGFRA (FIP1L1-PDGFRA) is a recurrent molecular lesion in eosinophilia-associated myeloproliferative disorders.

Subunit:
Component of the cleavage and polyadenylation specificity factor (CPSF) complex, composed of CPSF1, CPSF2, CPSF3, CPSF4 and FIP1L1. Found in a complex with CPSF1, FIP1L1 and PAPOLA. Interacts with CPSF1, CPSF4, CSTF2, CSTF3 and PAPOLA. 

Subcellular Location:
Nuclear

DISEASE:
Note=A chromosomal aberration involving FIP1L1 is found in some cases of hypereosinophilic syndrome. Interstitial chromosomal deletion del(4)(q12q12) causes the fusion of FIP1L1 and PDGFRA (FIP1L1-PDGFRA). 

Similarity:
Belongs to the FIP1 family. 

Database links:
UniProtKB/Swiss-Prot: Q6UN15.1

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

 

裂解和聚腺苷酸化特异性因子（CPSF）复合物的组成在前mRNA 3′端形成中起重要作用。该复合物识别AAUAAA信号序列并与聚（A）聚合酶相互作用以处理并添加到聚（A）尾。FIP1L1(FIP1-like 1)，也称为前mRNA 3’-末端加工因子FIP1、FIP1(与PAP相互作用的因子)和RHE(重排于嗜酸性粒细胞增多症)，是一种594个氨基酸的核蛋白，是CPSF复合物的组成部分。在配合物中，FIP1L1有助于聚（A）识别并刺激聚（A）加成。由于4q12号染色体上的间质缺失导致的FIP1L1和PDGFRA基因的融合是嗜酸性粒细胞综合征的原因，这是一种罕见的血液疾病，其特征是骨髓中嗜酸性粒细胞的持续过量生产导致组织浸润和器官损伤。有三种异构体的FIP1L1，作为替代剪接事件的结果。