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  4. Recombinant Human Midline-1 (MID1)

Recombinant Human Midline-1 (MID1)

  • 货号：
    CSB-YP013819HU
  • 规格：
  • 来源：
    Yeast
  • 其他：
  • 货号：
    CSB-EP013819HU
  • 规格：
  • 来源：
    E.coli
  • 其他：
  • 货号：
    CSB-EP013819HU-B
  • 规格：
  • 来源：
    E.coli
  • 共轭：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 货号：
    CSB-BP013819HU
  • 规格：
  • 来源：
    Baculovirus
  • 其他：
  • 货号：
    CSB-MP013819HU
  • 规格：
  • 来源：
    Mammalian cell
  • 其他：

产品详情

  • 纯度：
    >85% (SDS-PAGE)
  • 基因名：
  • Uniprot No.：
  • 别名：
    BBBG 1; BBBG1; E3 ubiquitin-protein ligase Midline-1; Finger on X and Y mouse homolog of; FXY; GBBB 1; GBBB1; 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; OSX; Putative transcription factor XPRF; RING finger protein 59; RING finger protein Midline-1; 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 on X and Y, mouse, homolog of; Zinc finger X and Y; ZNFXY
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    Full length protein
  • 表达区域：
    1-667
  • 氨基酸序列
    METLESELTC PICLELFEDP LLLPCAHSLC FNCAHRILVS HCATNESVES ITAFQCPTCR HVITLSQRGL DGLKRNVTLQ NIIDRFQKAS VSGPNSPSET RRERAFDANT MTSAEKVLCQ FCDQDPAQDA VKTCVTCEVS YCDECLKATH PNKKPFTGHR LIEPIPDSHI RGLMCLEHED EKVNMYCVTD DQLICALCKL VGRHRDHQVA ALSERYDKLK QNLESNLTNL IKRNTELETL LAKLIQTCQH VEVNASRQEA KLTEECDLLI EIIQQRRQII GTKIKEGKVM RLRKLAQQIA NCKQCIERSA SLISQAEHSL KENDHARFLQ TAKNITERVS MATASSQVLI PEINLNDTFD TFALDFSREK KLLECLDYLT APNPPTIREE LCTASYDTIT VHWTSDDEFS VVSYELQYTI FTGQANVVSL CNSADSWMIV PNIKQNHYTV HGLQSGTKYI FMVKAINQAG SRSSEPGKLK TNSQPFKLDP KSAHRKLKVS HDNLTVERDE SSSKKSHTPE RFTSQGSYGV AGNVFIDSGR HYWEVVISGS TWYAIGLAYK SAPKHEWIGK NSASWALCRC NNNWVVRHNS KEIPIEPAPH LRRVGILLDY DNGSIAFYDA LNSIHLYTFD VAFAQPVCPT FTVWNKCLTI ITGLPIPDHL DCTEQLP
  • 蛋白标签：
    Tag type will be determined during the manufacturing process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 产品提供形式：
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 复溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 货期：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

产品评价

靶点详情

  • 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.
  • 基因功能参考文献：
    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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  • 相关疾病：
    Opitz GBBB syndrome 1 (GBBB1)
  • 亚细胞定位：
    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
  • 组织特异性：
    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.
  • 数据库链接：

    HGNC: 7095

    OMIM: 300000

    KEGG: hsa:4281

    STRING: 9606.ENSP00000312678

    UniGene: Hs.27695