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Product Name FXR2 Chinese Name 脆性X相关蛋白样2抗体 Alias FMR1L2; Fragile X mental retardation 1 like 2; Fragile X mental retardation autosomal homolog 2; Fragile X mental retardation gene autosomal homolog 2; Fragile X mental retardation syndrome related protein 2; Fragile X mental retardation syndrome-related protein 2; FXR 2; FXR2; FXR2 PEN; FXR2_HUMAN; Human fragile X mental retardation syndrome related protein FXR2 mRNA complete cds. Research Area Cell biology immunology Neurobiology Epigenetics Immunogen Species Rabbit Clonality Polyclonal React Species Rat, (predicted: Human, Mouse, Chicken, Dog, Pig, Cow, Horse, Rabbit, ) Applications ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 74kDa Cellular localization cytoplasmic Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human FXR2: 261-360/673 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail Fragile X syndrome is caused by the absence of the fragile X mental-retardation protein (FMRP). FMRP is the archetype of a class of cytoplasmic mRNA-binding proteins that includes the fragile X-related 1 and 2 proteins (FXR1 and FXR2). The fragile X-related proteins FXR1 and FXR2 contain a functional nucleolar-targeting signal equivalent to the HIV-1 regulatory proteins.
Function:
RNA-binding protein.
Subunit:
Interacts with FMR1 and FXR1. Interacts with CYFIP2 but not with CYFIP1. Interacts with TDRD3.
Subcellular Location:
Cytoplasm.
Similarity:
Belongs to the FMR1 family.
Contains 2 Agenet-like domains.
Contains 2 KH domains.
SWISS:
Q06787
Gene ID:
9513
Database links:Entrez Gene: 9513 Human
Entrez Gene: 100343838 Rabbit
Omim: 605339 Human
SwissProt: P51116 Human
Unigene: 52788 Human
脆性X综合症,又称马丁-贝尔综合症,是一种遗传疾病。该综合症可以导致一系列的特征性症状,包括生理、智力、情绪、以及行为上的异常。症状的轻重各有不同。该疾病伴随着X染色体上一个简单的三核苷酸基因序列(CGG)的扩增。这种扩增导致了一种称为FMR-1的蛋白质无法在病人体内表达,而该蛋白质是神经的正常发育必不可少的。
根据CGG重复序列的长度,目前普遍认可将脆性X综合症分为四种类型:正常人(含有19-31个CGG重复序列),前突变者(含有55-200个CGG重复序列),全突变者(含有200个以上的CGG重复序列),过渡型,又称“灰色区域型”(含有40-60个重复)。脆性X综合征这是一种导致智力低下的遗传疾病,是导致人群中智力低下的第二大病因——仅次于21三体综合症。Product Picture Paraformaldehyde-fixed, paraffin embedded (rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (FXR2) Polyclonal Antibody, Unconjugated (SL6975R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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