FITC标记的肌肉特异性蛋白MYOZ2抗体
产品名称： FITC标记的肌肉特异性蛋白MYOZ2抗体
英文名称： Anti-Myozenin 2/FITC
产品编号： HZ-18975R-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-Myozenin 2/FITC Conjugated antibody
FITC标记的肌肉特异性蛋白MYOZ2抗体
| 英文名称 | Anti-Myozenin 2/FITC |
| 中文名称 | FITC标记的肌肉特异性蛋白MYOZ2抗体 |
| 别 名 | C4orf5; Calcineurin binding protein calsarcin 1; Calsarcin 1; Calsarcin-1; Calsarcin1; CMH16; CS 1; CS1; FATZ related protein 2; FATZ-related protein 2; Muscle specific protein; MYOZ 2; MYOZ2; MYOZ2_HUMAN; Myozenin-2; Myozenin2. |
| 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 信号转导 细胞骨架 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, 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 | 30kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Myozenin 2 |
| 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 protein encoded by this gene belongs to a family of sarcomeric proteins that bind to calcineurin, a phosphatase involved in calcium-dependent signal transduction in diverse cell types. These family members tether calcineurin to alpha-actinin at the z-line of the sarcomere of cardiac and skeletal muscle cells, and thus they are important for calcineurin signaling. Mutations in this gene cause cardiomyopathy familial hypertrophic type 16, a hereditary heart disorder. [provided by RefSeq, Aug 2011] Function: Myozenins may serve as intracellular binding proteins involved in linking Z line proteins such as alpha-actinin, gamma-filamin, TCAP/telethonin, LDB3/ZASP and localizing calcineurin signaling to the sarcomere. Plays an important role in the modulation of calcineurin signaling. May play a role in myofibrillogenesis. Subcellular Location: Cytoplasm > myofibril > sarcomere > Z line. Colocalizes with ACTN1 and PPP3CA at the Z-line of heart and skeletal muscle. Tissue Specificity: Expressed specifically in heart and skeletal muscle. DISEASE: Defects in MYOZ2 are the cause of familial hypertrophic cardiomyopathy type 16 (CMH16) [MIM:613838]. CMH16 is a hereditary heart disorder characterized by ventricular hypertrophy, which is usually asymmetric and often involves the interventricular septum. The symptoms include dyspnea, syncope, collapse, palpitations, and chest pain. They can be readily provoked by exercise. The disorder has inter- and intrafamilial variability ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death. Similarity: Belongs to the myozenin family. Database links: Entrez Gene: 51778 Human Entrez Gene: 540487 Cow Entrez Gene: 59006 Mouse Entrez Gene: 295426 Rat Entrez Gene: 100526817 Sheep Omim: 605602 Human SwissProt: Q5E9V3 Cow SwissProt: Q9NPC6 Human SwissProt: Q9JJW5 Mouse Unigene: 732122 Human Unigene: 141157 Mouse Unigene: 12931 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
该基因编码的蛋白属于与钙调神经磷酸酶（calcineurin）结合的肉瘤蛋白家族，钙调神经磷酸酶参与多种细胞类型的钙依赖性信号转导。这些家族成员将钙调神经磷酸酶和α-肌动蛋白在心肌和骨骼肌细胞的肌节的z线处结合在一起，因此它们对钙调神经磷酸酶信号转导很重要。这种基因突变导致心肌病家族性肥厚型16型，遗传性心脏病。[ RefSeq，Aug 2011提供]