环指蛋白59抗体
规格：1mg/1ml
英文名： RNF59/MID1
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 prote
分子量： 75kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human MID1/Mid
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Horse, Sheep,
细胞定位：细胞浆
环指蛋白59抗体产品介绍：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. 环指蛋白59抗体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 *****, 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. Gene ID: 4281 Database links: Entrez Gene: 4281 Human Entrez Gene: 17318 Mouse Entrez Gene: 54252 Rat Omim: 300552 Human SwissProt: 环指蛋白59抗体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.
环指蛋白59抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 **学 锌指蛋白
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid
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