FITC标记的盲肌样RNA结合蛋白MBNL2抗体
产品名称： FITC标记的盲肌样RNA结合蛋白MBNL2抗体
英文名称： Anti-MBNL2/FITC
产品编号： HZ-18707R-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-MBNL2/FITC Conjugated antibody
FITC标记的盲肌样RNA结合蛋白MBNL2抗体
| 英文名称 | Anti-MBNL2/FITC |
| 中文名称 | FITC标记的盲肌样RNA结合蛋白MBNL2抗体 |
| 别 名 | DKFZp781H1296; MBLL; MBLL39; Mbnl2; MBNL2_HUMAN; MGC120625; MGC120626; MGC120628; Muscleblind like 2; muscleblind like protein 1; muscleblind like protein 2; muscleblind like protein like 39; muscleblind like splicing regulator 2; Muscleblind-like protein 1; Muscleblind-like protein 2; Muscleblind-like protein-like 39; Muscleblind-like protein-like; OTTHUMP00000178763; PRO2032; RP11-128N14.1. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 结合蛋白 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Chicken, Dog, Cow, Horse, 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 | 41kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human MBNL2 |
| 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: This gene is a member of the muscleblind protein family which was initially described in Drosophila melanogaster. This gene encodes a C3H-type zinc finger protein that modulates alternative splicing of pre-mRNAs. Muscleblind proteins bind specifically to expanded dsCUG RNA but not to normal size CUG repeats and may thereby play a role in the pathophysiology of myotonic dystrophy. Several alternatively spliced transcript variants have been described but the full-length natures of only some have been determined. [provided by RefSeq, Mar 2012] Function: Mediates pre-mRNA alternative splicing regulation. Acts either as activator or repressor of splicing on specific pre-mRNA targets. Inhibits cardiac troponin-T (TNNT2) pre-mRNA exon inclusion but induces insulin receptor (IR) pre-mRNA exon inclusion in muscle. Antagonizes the alternative splicing activity pattern of CELF proteins. RNA-binding protein that binds to 5'ACACCC-3' core sequence, termed zipcode, within the 3'UTR of ITGA3. Binds to CUG triplet repeat expansion in myotonic dystrophy muscle cells by sequestering the target RNAs. Seems to regulates expression and localization of ITGA3 by transporting it from the nucleus to cytoplasm at adhesion plaques. May play a role in myotonic dystrophy pathophysiology (DM). Subcellular Location: Nucleus. Cytoplasm. Greater concentration in the nucleus. Expressed in or near large cytoplasmic adhesion plaques. Location in the cytoplasm is microtubule-dependent. In both DM1 and DM2 patients, colocalizes with nuclear foci of retained expanded-repeat transcripts. Tissue Specificity: Expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas. Similarity: Belongs to the muscleblind family. Contains 4 C3H1-type zinc fingers. Database links: Entrez Gene: 10150 Human Entrez Gene: 105559 Mouse Entrez Gene: 680445 Rat Omim: 607327 Human SwissProt: Q5VZF2 Human SwissProt: Q8C181 Mouse SwissProt: F2Z3T4 Rat Unigene: 657347 Human Unigene: 238266 Mouse Unigene: 1940 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic application |
该基因是最初在Drosophila melanogaster中描述的肌盲蛋白家族的成员。该基因编码C3H型锌指蛋白，其调节前mRNA的选择性剪接。肌盲蛋白与扩张的dsCUG RNA特异性结合，但不与正常大小的CUG重复序列结合，因此可能在强直性肌营养不良的病理生理学中发挥作用。已经描述了几种选择性剪接的转录体变体，但是仅确定了一些的全长性质。〔RefSeq，2012〕