FITC标记的环指蛋白59抗体
产品名称： FITC标记的环指蛋白59抗体
英文名称： Anti-RNF59/MID1/FITC
产品编号： HZ-9380R-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-RNF59/MID1/FITC Conjugated antibody
FITC标记的环指蛋白59抗体
| 产品编号 | bs-9380R-FITC |
| 英文名称 | Anti-RNF59/MID1/FITC |
| 中文名称 | FITC标记的环指蛋白59抗体 |
| 别 名 | BBBG 1; BBBG1; Finger on X and Y mouse homolog of antibody; FXY; GBBB 1; GBBB1; MID 1; MID-1; Mid1; Midin; Midline 1 (Opitz/BBB syndrome); Midline 1; Midline 1 ring finger; Midline 1 RING finger protein; Midline-1; Midline1; OGS 1; OGS1; OS antibody; OSX; Putative transcription factor XPRF; RING finger protein 59; RNF 59; RNF59; TRI18; TRI18_HUMAN; TRIM 18; TRIM18; Tripartite motif containing protein 18; Tripartite motif protein TRIM18; Tripartite motif-containing protein 18; XPRF; Zinc finger X and Y antibody; ZNFXY. |
| 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 免疫学 锌指蛋白 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Chicken, Dog, Pig, 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 | 75kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human MID1/Midline-1/RNF59 |
| 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: Midline-1 (Tripartite motif-containing protein 18, Putative transcription factor XPRF, RING finger protein 59) is a 667 amino acid protein encoded by the human gene MID1. Midline-1 belongs to the TRIM/RBCC family and contains two B box-type zinc fingers, one B30.2/SPRY domain, one COS domain, one fibronectin type-III domain and one RING-type zinc finger. Midline-1 is believed to have E3 ubiquitin ligase activity which targets the catalytic subunit of protein phosphatase 2 for degradation. It is a cytoplasmic protein found as a homodimer or heterodimer with Midline-2. It also interacts with IGBP1 (Lymphocyte signaling protein A4). Defects in MID1 are the cause of Opitz syndrome type I (OS-I). OS-I is an X-linked recessive disorder characterized by hypertelorism, genital-urinary defects such as hypospadias in males and splayed labia in females, lip-palate-laryngotracheal clefts, imperforate anus, developmental delay and congenital heart defects. OS-I mutations produce proteins with a decreased affinity for microtubules. Function: Has E3 ubiquitin ligase activity towards IGBP1, promoting its monoubiquitination, which results in deprotection of the catalytic subunit of protein phosphatase PP2A, and its subsequent degradation by polyubiquitination. Subunit: Homodimer or heterodimer with MID2. Interacts with IGBP1. Subcellular Location: Cytoplasm. Tissue Specificity: In the fetus, highest expression found in kidney, followed by brain and lung. Expressed at low levels in fetal liver. In the adult, most abundant in heart, placenta and brain. Post-translational modifications: Phosphorylated on serine and threonine residues. DISEASE: Defects in MID1 are the cause of Opitz GBBB syndrome 1 (OGS1) [MIM:300000]. A congenital midline malformation syndrome characterized by hypertelorism, genital-urinary defects such as hypospadias in males and splayed labia in females, lip-palate-laryngotracheal clefts, imperforate anus, developmental delay and congenital heart defects. Note=MID1 mutations produce proteins with a decreased affinity for microtubules. Similarity: Belongs to the TRIM/RBCC family. Contains 2 B box-type zinc fingers. Contains 1 B30.2/SPRY domain. Contains 1 COS domain. Contains 1 fibronectin type-III domain. [SIMILARITY] Contains 1 RING-type zinc finger. Database links: Entrez Gene: 4281 Human Entrez Gene: 17318 Mouse Entrez Gene: 54252 Rat Omim: 300552 Human SwissProt: O15344 Human SwissProt: O70583 Mouse SwissProt: P82458 Rat Unigene: 27695 Human Unigene: 689953 Human Unigene: 34441 Mouse Unigene: 444905 Mouse Unigene: 460870 Mouse Unigene: 15169 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
MIDLIE -1（三分体基序蛋白18，推测转录因子XPRF，环指蛋白59）是由人类基因MID1编码的667氨基酸蛋白。MIDLIE -1属于Trim/RBCC家族，包含两个B盒型锌指，一个B30.2/SPRY结构域，一个COS结构域，一个纤连蛋白Ⅲ型结构域和一个环状锌指结构。MIDLIE -1被认为具有靶向蛋白磷酸酶2的催化亚基的E3泛素连接酶活性，用于降解。它是一种细胞质蛋白，被发现为中二聚体或异源二聚体。它也与IGBP1（淋巴细胞信号蛋白A4）相互作用。MID1中的缺陷是I型OPSZ综合征（OS-Ⅰ）的病因。OS-Ⅰ是一种X连锁隐性遗传病症，其特征是肥大、女性尿道下裂、女性唇裂、唇腭裂、肛门闭锁、发育迟缓和先天性心脏病。OS-Ⅰ突变产生与微管亲和力降低的蛋白质。