脆性X综合征相关蛋白AFF2抗体

英文名称    AFF2    

中文名称    脆性X综合征相关蛋白AFF2抗体    

别    名    FMR2; AF4/FMR2 family member 2; AF4/FMR2 family, member 2; AFF2; AFF2_HUMAN; FMR2; FMR2P; Fragile X E mental retardation syndrome protein; fragile X mental retardation 2; Fragile X mental retardation 2 protein; fragile X mental retardation gene associated with FRAXE; FRAXE; mild or borderline mental retardation; MRX2; OX19; Protein FMR-2; Protein Ox19.    

说 明 书    0.1ml  0.2ml      

研究领域    神经生物学      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Dog, Pig, Cow, Horse,     

产品应用    WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 脆性X综合征相关蛋白AFF2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    145kDa    

细胞定位    细胞核     

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human AFF2 (1-80aa)    

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.    

脆性X综合征相关蛋白AFF2抗体产品介绍    background:

FMR2 is a 1311 amino acid nuclear protein belonging to the AF4 family. Expressed in the brain, placenta and lung, FMR2 exists as two isoforms produced by alternative splicing. Defects in the gene that encodes FMR2 have been found to be a cause of FRAXE, an X-linked form of mental retardation. Individuals expressing the FRAXE site also have more than two-hundred copies of a GCC repeat adjacent to CpG island, compared to six to thirty-five copies of the GCC repeat in a normal individual. It is believed that loss of FMR2 expression causes this GCC expansion of the FRAXE site. 

Function:
RNA-binding protein. Might be involved in alternative splicing regulation through an interaction with G-quartet RNA structure.

Subcellular Location:
Nucleus speckle. When splicing is inhibited, accumlates in enlarged speckles.

Tissue Specificity:
Brain (most abundant in hippocampus and amygdala), placenta and lung.

DISEASE:
Defects in AFF2 are the cause of fragile X-E mental retardation syndrome (FRAXE) [MIM:309548]. FRAXE is an X-linked form of mental retardation. Loss of FMR2 expression is correlated with FRAXE CCG(N) expansion. Normal individuals have 6-35 copies of the repeat, whereas cytogenetically positive, developmentally delayed males have more than 200 copies and show methylation of the associated CPG island.

Similarity:
Belongs to the AF4 family.

Database links:

Entrez Gene: 2334 Human

Omim: 309548 Human

SwissProt: P51816 Human

Unigene: 496911 Human

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

脆性X综合征相关蛋白AFF2抗体

hz-0812R IL-1 Beta/IL-1B  白介素1β抗体 IL-1β

hz-1191R IL-2  白介素2抗体（大、小鼠）

hz-4586R IL-2  白介素2抗体

hz-4586M IL-2  白介素2抗体

hz-0605R IL-2  白介素2抗体(人)

hzm-0389M IL-2(H2E4)  白细胞介素-2单克隆抗体

hz-2784R ITK/PSCTK2  IL-2诱导型T细胞激酶抗体

hz-2598R IL-3  白介素3抗体

hz-4510R IL-3(human)  白介素3抗体（人）

hz-2600R IL-3R alpha/CD123  白介素3受体a链抗体

hz-0581R IL-4  白介素4抗体

hz-1740R IL-4 (human)  白介素4抗体(人)

hz-2458R IL-4R/CD124  白细胞介素4受体抗体IL-4Rα

hz-1318R IL-5  白细胞介素5抗体

hz-2601R IL5RA/CD125  白介素-5受体α链抗体IL-5Rα

hz-0781R IL-6  白介素6

hz-4539R IL-6  白介素6

hz-4587M IL-6  白介素6抗体

hz-0782R IL-6  白介素6抗体

hz-4540R IL-6 (NT)  白介素6抗体(N端)

hz-0379R IL-6  白介素6抗体

hz-1811R IL-7  白介素7抗体

hz-1805R IL-6R alpha  白细胞介素6受体α抗体IL-6Rα

hz-1459R IL-6R Beta/CD130/gp130  白细胞介素6受体β抗体IL-6Rβ

hz-0780R IL-8/CXCL8  白介素8抗体

hz-1540R IL-7Ra/CD127  白细胞介素-7受体a抗体

hz-5395R phospho-IL-7Ra(Tyr449)  磷酸化白细胞介素-7受体a抗体

hz-1824R Mitofilin/IMMT  线粒体内膜蛋白抗体

hz-0317R ILK-1  整合素连接激酶-1抗体

hz-5443R phospho-ILK-1(Thr173)  磷酸化整合素连接激酶1抗体

hz-5444R phospho-ILK-1(Ser246)   磷酸化整合素连接激酶1抗体

hz-5445R phospho-ILK-1(Ser259)  磷酸化整合素连接激酶1抗体

hz-1291R ING1/p33  生长抑制因子基因1抗体

hz-1521R IGF2BP3/IMP3  胰岛素样生长因子ⅡmRNA结合蛋白3抗体