产品中心
当前位置：首页>产品中心Anti-RNF59/MID1/FITC
货号： bs-9380R-FITC 基本售价： 2980.0 元 规格： 100ul
产品信息
- 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.