MID1 Antibody

說明書

中文名稱：

MID1兔多克隆抗體
貨號：

CSB-PA003239
規格：

￥1090
圖片：
Western Blot analysis of 293 cells using Midline-1 Polyclonal Antibody

Western Blot analysis of 293 cells using Midline-1 Polyclonal Antibody
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

O15344
基因名：

MID1
別名：

BBBG 1 antibody; BBBG1 antibody; E3 ubiquitin-protein ligase Midline-1 antibody; Finger on X and Y mouse homolog of antibody; FXY antibody; GBBB 1 antibody; GBBB1 antibody; MID 1 antibody; Mid1 antibody; Midin antibody; Midline 1 (Opitz/BBB syndrome) antibody; Midline 1 antibody; Midline 1 ring finger antibody; Midline 1 RING finger protein antibody; Midline-1 antibody; Midline1 antibody; OGS 1 antibody; OGS1 antibody; OS antibody; OSX antibody; Putative transcription factor XPRF antibody; RING finger protein 59 antibody; RING finger protein Midline-1 antibody; RNF 59 antibody; RNF59 antibody; TRI18 antibody; TRI18_HUMAN antibody; TRIM 18 antibody; TRIM18 antibody; Tripartite motif containing protein 18 antibody; Tripartite motif protein TRIM18 antibody; Tripartite motif-containing protein 18 antibody; XPRF antibody; zinc finger on X and Y, mouse, homolog of antibody; Zinc finger X and Y antibody; ZNFXY antibody
宿主：

Rabbit
反應種屬：

Human,Mouse,Rat
免疫原：

Synthesized peptide derived from the N-terminal region of Human Midline-1.
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
抗體亞型：

IgG
純化方式：

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
產品提供形式：

Liquid
應用范圍：

WB, IHC, IF, ELISA

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:2000
IHC	1:100-1:300
IF	1:200-1:1000
ELISA	1:40000

Protocols：

Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
Immunofluorescence (IF) Protocol
ELISA Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

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.
基因功能參考文獻：
1. Data show that protein phosphatase-2A (PP2A) was upregulated in lung adenocarcinoma cell lines that were transfected with midline 1 E3 ubiquitin-protein ligase (MID1)-siRNA, suggesting MID1 negatively regulates PP2A in lung adenocarcinoma. PMID: 29450633
2. identified four miRNAs, miR-19, miR-340, miR-374 and miR-542 that bind to the 3'-UTR of the MID1 mRNA. These miRNAs not only regulate MID1 expression but also mTOR signaling and translation of disease associated mRNAs and could therefore serve as potential drugs for future therapy development PMID: 29293623
3. Our data reveal a novel role for MID1 and for atypical ubiquitination in balancing BRAF35 presence, and likely its activity, within nuclear and cytoplasmic compartments PMID: 28760657
4. P151L MID1 mutation is associated with X-linked Opitz Syndrome. PMID: 28548391
5. the coiled-coil and COS domain (CC-COS) bind to microtubules, demonstrating for the first time that MID1 can directly associate with the microtubules PMID: 27367845
6. Osx is upregulated in patients with Ossification of the posterior longitudinal ligament. PMID: 27693496
7. A130T/V mutations within the MID1 zinc-binding Bbox1 domain affects protein folding. PMID: 25874572
8. MID1 catalyzes the ubiquitination of protein phosphatase 2A and mutations within its Bbox1 domain disrupt polyubiquitination of alpha4 but not of PP2Ac in X-linked Opitz syndrome. PMID: 25207814
9. TRAIL regulates MID1 and TSLP, inflammation, fibrosis, smooth muscle hypertrophy, and expression of inflammatory effector chemokines and cytokines in experimental eosinophilic esophagitis. PMID: 25981737
10. These studies provide insight into the mechanism by which mutations observed in X-linked Opitz G syndrome affect the structure and function of the MID1 Bbox1 domain PMID: 25216264
11. A familial c.1102C>T (p.R368X) mutation in the MID1 gene, is reported. PMID: 25304119
12. Results revealed S422 as a novel phosphorylation site of Osx and GSK-3b played an important role in regulating the protein stability and transactivational activity of Osx. PMID: 25728276
13. Fu ubiquitination and cleavage is one of the key elements connecting the MID1-PP2A protein complex with GLI3 activity control PMID: 25278022
14. Promotion of AR, in addition to enhancement of the Akt-, NFkappaB-, and Hh-pathways by sustained MID1-upregulation during androgen deprivation therapy provides a powerful proliferative scenario for PCa progression into castration resistance PMID: 24913494
15. In prostate cancer cells the inhibitory effect of metformin was mimicked by disruption of MID1 translational regulator complex. PMID: 24484909
16. Two patients with underdeveloped arcuate fasciculus had novel, nonsynonymous variants in MID1 and EN2 genes regulating axon guidance pathway. PMID: 24321989
17. for the first time within the MID1 gene, a complex rearrangement composed of two deletions, an inversion and a small insertion that may suggest the involvement of concurrent non-homologous mechanisms in the generation of the observed structural variant. PMID: 23791568
18. expanded CAG repeats bind to a translation regulatory protein complex containing MID1, protein phosphatase 2A and 40S ribosomal S6 kinase. PMID: 23443539
19. found that MID1 was upregulated in primary human bronchial epithelial cells PMID: 23334847
20. Protein phosphatase 2A (PP2A)-specific ubiquitin ligase MID1 is a sequence-dependent regulator of translation efficiency controlling 3-phosphoinositide-dependent protein kinase-1 (PDPK-1). PMID: 21930711
21. mTORC1 signaling as a downstream pathway regulated by the MID1/PP2A axis, suggesting that mTORC1 plays a key role in Opitz syndrome pathogenesis. PMID: 21555591
22. Our finding suggests that hypospadias associated with hypertelorism is the mildest phenotype in OS caused by MID1 mutations. PMID: 21326312
23. These studies shed light on MID1 E3 ligase activity and show how its three zinc-binding domains can contribute to MID1's overall function. PMID: 21296087
24. MID1 coiled-coil motifs mediate both homo- and heterodimerization, a prerequisite for association of the MID-alpha 4 complex with microtubules. PMID: 11806752
25. These searches revealed a fusion transcript containing the LTR of an HERV-E element linked to the Opitz syndrome gene Mid1 PMID: 12411602
26. identified a duplication of the MID1 first exon in a patient with X-linked Opitz G/BBB syndrome PMID: 12545276
27. The Opitz syndrome gene MID1 is essential for establishing asymmetric gene expression in Hensen's node. PMID: 12798296
28. Novel mutations are described in the MID1 gene, particularly in the 3' flanking region. PMID: 12833403
29. FibronectinIII domain of the MID1 protein may be involved in midline differentiation after neural tube and palatal structures are completed. PMID: 16378742
30. a novel insertion mutation (c.1798_1799-insC)in MID1 gene was identified; first report on a genetically confirmed case of X-linked Opitz G/BBB syndrome in Korea PMID: 17043407
31. Twenty-nine novel mutations are associated with Opitz G/BBB Syndrome. PMID: 17221865
32. The tertiary structure of the B-box2 (CHC(D/C)C(2)H(2)) domain from MID1, is reported using multidimensional nuclear magnetic resonance spectroscopy. PMID: 17428496
33. MID1 expression is tightly regulated through concerted action of alternative promoters and alternative polyadenylation signals both during embryonic development and in the adult. PMID: 18005432
34. Mutant MID1 proteins cannot bind EF-1alpha and results in the development of the ventral midline in Opitz syndrome patients PMID: 18172692
35. Structure of the MID1 tandem B-boxes reveals an interaction reminiscent of intermolecular ring heterodimers. PMID: 18220417
36. reviewed all the MID1 mutations reported so far, in both familial and sporadic cases. The mutations are scattered along the entire length of the gene PMID: 18360914
37. A new MID1 mutation leading to a premature stop codon was found in patients with Opitz G/BBB syndrome. PMID: 18697196
38. Active transport of the ubiquitin ligase MID1 along the microtubules is regulated by protein phosphatase 2A. PMID: 18949047
39. Non-random tranmission of MID1 haplotypes in male non-syndromic cleft lip with or without cleft palate patients was observed. PMID: 19049519
40. the apparent occurrence of an unusual TG 3' splice site in intron 9 is discussed PMID: 17672918
41. PP2Ac accumulation is caused by an impairment of a newly identified E3 ubiquitin ligase activity of the MID1 protein PMID: 11685209
42. Widely spaced alternative promoters, conserved between human and rodent, control the expression of Mid1 PMID: 12408967
43. Mid1 is transcribed from a human endogenous retroviral promoter PMID: 12411602
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相關疾病：

Opitz GBBB syndrome 1 (GBBB1)
亞細胞定位：

Cytoplasm. Cytoplasm, cytoskeleton. Cytoplasm, cytoskeleton, spindle. Note=Microtubule-associated. It is associated with microtubules throughout the cell cycle, co-localizing with cytoplasmic fibers in interphase and with the mitotic spindle and midbodies during mitosis and cytokinesis.
蛋白家族：

TRIM/RBCC family
組織特異性：

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.
數據庫鏈接：

HGNC: 7095

OMIM: 300000

KEGG: hsa:4281

STRING: 9606.ENSP00000312678

UniGene: Hs.27695

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