FITC标记的毒蕈碱样蛋白3抗体
产品名称： FITC标记的毒蕈碱样蛋白3抗体
英文名称： Anti-MBNL3/FITC
产品编号： HZ-5721R-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： IF=1:50-200
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Rabbit Anti-MBNL3/FITC Conjugated antibody
FITC标记的毒蕈碱样蛋白3抗体
| 英文名称 | Anti-MBNL3/FITC |
| 中文名称 | FITC标记的毒蕈碱样蛋白3抗体 |
| 别 名 | MBLX; CHCR; Cys3His CCG1 required protein; MBLX39; MBNL 3; MBXL; Muscleblind like protein 3; Muscleblind like X linked protein; Protein HCHCR; MBNL3_HUMAN. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 细胞生物 免疫学 染色质和核信号 细胞周期蛋白 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Pig, Cow, Horse, Sheep, |
| 产品应用 | IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 39kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human MBNL3 |
| 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: MBNL3 has been shown to inhibit muscle differentiation. MBNL3 belongs to a highly conserved family of tissue-specific alternative splicing regulators. This family is known to regulate terminal muscle differentiation through alternative splicing control and several groups have suggested that the family participates in the differentiation of photoreceptors, neurons, adipocytes and blood cell types. MBNL3 is expressed in myoblasts, muscle precursor cells, and during the early stages of myogenesis, but is detected at very low levels in terminally differentiated myotubes. MBNL proteins have been shown to sequester foci of expanded-repeat transcripts and are thought to therefore play a role in the molecular pathology of a group of neuromuscular diseases including the Myotonic Dystrophies. 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. May play a role in myotonic dystrophy pathophysiology (DM). Could inhibit terminal muscle differentiation, acting at approximately the time of myogenin induction. Subcellular Location: Nucleus. Cytoplasm. Note=Greater concentration in the nucleus. In both DM1 and DM2 patients, colocalizes with nuclear foci of retained expanded-repeat transcripts. Tissue Specificity: Highly expressed in the placenta. Similarity: Belongs to the muscleblind family. Contains 4 C3H1-type zinc fingers. Database links: Entrez Gene: 55796 Human Entrez Gene: 171170 Mouse Entrez Gene: 302492 Rat Omim: 300413 Human SwissProt: Q9NUK0 Human SwissProt: Q8R003 Mouse Unigene: 105134 Human Unigene: 596347 Human Unigene: 295324 Mouse Unigene: 105030 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
MnNL3已被证明可以抑制肌肉分化。MnNL3属于高度保守的组织特异性剪接调节子家族。该家族已知通过选择性剪接控制调节末梢肌肉分化，并且一些组已经建议该家族参与光感受器、神经元、脂肪细胞和血细胞类型的分化。MbnL3在成肌细胞、肌肉前体细胞和肌肉发育早期表达，但在终末分化的肌管中检测到非常低的水平。MNL蛋白已被证明隔离扩增重复转录灶的焦点，因此被认为是在一组神经肌肉疾病，包括强直性肌营养不良的分子病理学中发挥作用。