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Recombinant Human Ubiquilin-2 (UBQLN2)

  • 中文名稱：
    人UBQLN2重組蛋白
  • 貨號(hào)：
    CSB-YP883393HU
  • 規(guī)格：
  • 來源：
    Yeast
  • 其他：
  • 中文名稱：
    人UBQLN2重組蛋白
  • 貨號(hào)：
    CSB-EP883393HU
  • 規(guī)格：
  • 來源：
    E.coli
  • 其他：
  • 中文名稱：
    人UBQLN2重組蛋白
  • 貨號(hào)：
    CSB-EP883393HU-B
  • 規(guī)格：
  • 來源：
    E.coli
  • 共軛：
    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：
  • 中文名稱：
    人UBQLN2重組蛋白
  • 貨號(hào)：
    CSB-BP883393HU
  • 規(guī)格：
  • 來源：
    Baculovirus
  • 其他：
  • 中文名稱：
    人UBQLN2重組蛋白
  • 貨號(hào)：
    CSB-MP883393HU
  • 規(guī)格：
  • 來源：
    Mammalian cell
  • 其他：

產(chǎn)品詳情

  • 純度：
    >85% (SDS-PAGE)
  • 基因名：
  • Uniprot No.：
  • 別名：
    UBQLN2; N4BP4; PLIC2; HRIHFB2157Ubiquilin-2; Chap1; DSK2 homolog; Protein linking IAP with cytoskeleton 2; PLIC-2; hPLIC-2; Ubiquitin-like product Chap1/Dsk2
  • 種屬：
    Homo sapiens (Human)
  • 蛋白長度：
    Full Length of Mature Protein
  • 表達(dá)區(qū)域：
    2-624
  • 氨基酸序列
    AENGESSGP PRPSRGPAAA QGSAAAPAEP KIIKVTVKTP KEKEEFAVPE NSSVQQFKEA ISKRFKSQTD QLVLIFAGKI LKDQDTLIQH GIHDGLTVHL VIKSQNRPQG QSTQPSNAAG TNTTSASTPR SNSTPISTNS NPFGLGSLGG LAGLSSLGLS STNFSELQSQ MQQQLMASPE MMIQIMENPF VQSMLSNPDL MRQLIMANPQ MQQLIQRNPE ISHLLNNPDI MRQTLEIARN PAMMQEMMRN QDLALSNLES IPGGYNALRR MYTDIQEPML NAAQEQFGGN PFASVGSSSS SGEGTQPSRT ENRDPLPNPW APPPATQSSA TTSTTTSTGS GSGNSSSNAT GNTVAAANYV ASIFSTPGMQ SLLQQITENP QLIQNMLSAP YMRSMMQSLS QNPDLAAQMM LNSPLFTANP QLQEQMRPQL PAFLQQMQNP DTLSAMSNPR AMQALMQIQQ GLQTLATEAP GLIPSFTPGV GVGVLGTAIG PVGPVTPIGP IGPIVPFTPI GPIGPIGPTG PAAPPGSTGS GGPTGPTVSS AAPSETTSPT SESGPNQQFI QQMVQALAGA NAPQLPNPEV RFQQQLEQLN AMGFLNREAN LQALIATGGD INAAIERLLG SQPS
  • 蛋白標(biāo)簽：
    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.
  • 產(chǎn)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.
  • 復(fù)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.
  • 儲(chǔ)存條件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質(zhì)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.
  • 貨期：
    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.
  • 注意事項(xiàng)：
    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.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

  • 功能：
    Plays an important role in the regulation of different protein degradation mechanisms and pathways including ubiquitin-proteasome system (UPS), autophagy and the endoplasmic reticulum-associated protein degradation (ERAD) pathway. Mediates the proteasomal targeting of misfolded or accumulated proteins for degradation by binding (via UBA domain) to their polyubiquitin chains and by interacting (via ubiquitin-like domain) with the subunits of the proteasome. Plays a role in the ERAD pathway via its interaction with ER-localized proteins FAF2/UBXD8 and HERPUD1 and may form a link between the polyubiquitinated ERAD substrates and the proteasome. Involved in the regulation of macroautophagy and autophagosome formation; required for maturation of autophagy-related protein LC3 from the cytosolic form LC3-I to the membrane-bound form LC3-II and may assist in the maturation of autophagosomes to autolysosomes by mediating autophagosome-lysosome fusion. Negatively regulates the endocytosis of GPCR receptors: AVPR2 and ADRB2, by specifically reducing the rate at which receptor-arrestin complexes concentrate in clathrin-coated pits (CCPs).
  • 基因功能參考文獻(xiàn)：
    1. The genome of Drosophila contains a single UBQLN homolog (dUbqn) that shows high similarity to UBQLN1 and UBQLN2; therefore, the fly is a useful model for characterizing the role of UBQLN in vivo in neurological disorders affecting locomotion and learning abilities. PMID: 29247619
    2. Its PXX domain missense mutations linked to amyotrophic lateral sclerosis and atypical hereditary spastic paraplegia phenotype through defective HSP70-mediated proteolysis. PMID: 28716533
    3. Its mutation is a cause of amyotrophic lateral sclerosis in New Zealand. PMID: 27480424
    4. Ubiquilins are a family of chaperones for cytosolically exposed transmembrane domains and use ubiquitin to triage clients for degradation via coordinated intra- and intermolecular interactions. PMID: 27345149
    5. We analyzed mutations in the UBQLN2 gene in a Chinese cohort with sporadic ALS (sALS). A novel missense mutation was detected in one sALS patient. The p.M392V mutation substitutes a highly conserved residue, has not been reported in the population databases, and previously, at the same residue, a missense mutation p.M392I was detected in two Turkey ALS patients and was considered to be pathogenic. PMID: 28125704
    6. Frontotemporal dementia -linked mutations in gene ubiquilin 2 encoding autophagy adaptor proteins , indicate that impaired autophagy might cause Frontotemporal dementia. PMID: 27166223
    7. excess UBQLN2 is toxic rather than protective to neurons and that uncontrolled enhancement of UBQLN2 function is involved in UBQLN2 pathogenesis PMID: 27456931
    8. Ubiquilin-2 immunostaining - a new marker as a diagnostic supplement in urine cytology? PMID: 27168037
    9. UBQLN2 is specifically expressed in the urine of urothelial carcinoma patients. PMID: 26303000
    10. Results showed that UBQLN2 is selectively recruited to nuclear inclusions in Huntington's disease but not spinocerebellar ataxia type 3 PMID: 26141599
    11. These findings provide a molecular basis for the development of ALS/FTD-associated proteinopathy and establish novel therapeutic targets for ALS. PMID: 26944018
    12. Mutations in UBQLN2 gene cause dominant inheritance of amyotrophic lateral sclerosis due to Defective Proteasome Delivery. PMID: 26075709
    13. UBQLN2 may be a new molecular target for chemotherapeutics and a useful clinicopathological marker in human osteosarcoma. PMID: 25672654
    14. ALS-linked mutations in ubiquilin-2 or hnRNPA1 reduce interaction between ubiquilin-2 and hnRNPA1 PMID: 25616961
    15. A single putative mutation of UBQLN2 in a cohort of patients with front temporal lobar degeneration was found. PMID: 25179229
    16. Data indicate cognitive deficits in mutant ubiquilin 2 protein UBQLN2P497H transgenic mice. PMID: 25246588
    17. Causative mutation in the UBQLN2 gene is rare in Korean patients with either familial or sporadic ALS. PMID: 24684794
    18. As ubiquilin-2-positive inclusions are identified in brain, this mutant peptide predisposes to protein misfolding and accumulation. PMID: 24771548
    19. The P506S mutation in UBQLN2 can affect both males and females with frontotemporal dementia/amyotrophic lateral sclerosis (FTD/ALS). PMID: 23944734
    20. no evidence for involvement of ubiquilin 2 in tau pathology PMID: 24086754
    21. The dtat of study suggest that UBQLN2 is generally involved in the pathogenesis of ALS. PMID: 24085347
    22. Its mutations are not frequent cause of amyotrophic lateral sclerosis in Ireland. PMID: 23973441
    23. results were confirmed by similar findings for ubiquilin-1 and -2 in human brain tissue sections, where accumulation was observed in huntingtin inclusions PMID: 23774650
    24. Genetic variations in UBQLN2 in a predominantly Flanders-Belgian cohort of frontotemporal lobar degeneration patients are extremely rare. PMID: 23312802
    25. Its mutations related to ALS/FTLD are extremely rare in French FTLD and FTLD-ALS patients. PMID: 23582661
    26. No causative mutations within the PXX domain of the UBQLN2 gene are found in familial frontotemoral dementia patients. PMID: 22729385
    27. data support the role of the UBQLN2 gene in the pathogenesis of FALS, being conversely a rare genetic cause in SALS even when complicated by FTD. PMID: 23138764
    28. This study reported 3 novel UBQLN2 mutations, accounting for 1.2% (2/161) ALS and 2.2% (1/45) FTD patients, including a patient with pure FTD. PMID: 22892309
    29. A novel missense UBQLN2 mutation (c.1460C>T, p.T487I) was identified in 2 apparently unrelated multigenerational amyotrophic lateral sclerosis families with no evidence of frontotemporal dementia. This mutation segregated with the disease. PMID: 22717235
    30. The results of this study support support a causative role of the UBQLN2 gene in the pathogenesis of ALS and suggest that UBQLN2 mutations are rare in the French and French-Canadian population. PMID: 22560112
    31. The results of this study suggested that UBQLN2 was not found to be a cause of familial ALS in the Netherlands. PMID: 22676852
    32. Found a pathophysiological link between C9ORF72 expansions and ubiquilin-2 (UBQLN) proteins in amyotrophic lateral sclerosis and frontotemporal lobar degeneration that is associated with a highly characteristic pattern of UBQLN pathology. PMID: 22426854
    33. The results of this study suggested that UBQLN2 gene mutations are rare in French amyotrophic lateral sclerosis. PMID: 22169395
    34. findings link abnormalities in ubiquilin 2 to defects in the protein degradation pathway, abnormal protein aggregation and neurodegeneration, indicating a common pathogenic mechanism that can be exploited for therapeutic intervention PMID: 21857683
    35. solution structure of the ubl domain of hPLIC-2 PMID: 11827521
    36. hPLIC-2 interferes with the ubiquitin-mediated proteolysis of p53 and interacts with proteasomes PMID: 12972570
    37. Ubiquilin is capable of forming dimers. Dimerization requires the central region of ubiquilin, but not its UBL or the UBA domains. Monomeric ubiquilin is likely to be the active form that is involved in binding presenilin proteins. PMID: 16813565
    38. hHR23a and hPLIC2 interact via UBL/UBA domain interactions PMID: 17098253
    39. Ubiquitin-like protein PLIC-2 is identified as a negative regulator of G protein-coupled receptor endocytosis. PMID: 18199683
    40. siRNA-mediated UBQLN2 depletion made cells more susceptible to starvation-induced cell death. UBQLN2 regulates cell survival during starvation. PMID: 19148225

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  • 相關(guān)疾病：
    Amyotrophic lateral sclerosis 15, with or without frontotemporal dementia (ALS15)
  • 亞細(xì)胞定位：
    Cytoplasm. Nucleus. Membrane. Cytoplasmic vesicle, autophagosome.
  • 數(shù)據(jù)庫鏈接：

    HGNC: 12509

    OMIM: 300264

    KEGG: hsa:29978

    STRING: 9606.ENSP00000345195

    UniGene: Hs.179309