Recombinant Human Double homeobox protein 4(DUX4),partial

Code CSB-YP890667HU1
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Source Yeast
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Code CSB-EP890667HU1
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Source E.coli
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Code CSB-EP890667HU1-B
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Source E.coli
Conjugate 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.
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Code CSB-BP890667HU1
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Source Baculovirus
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Code CSB-MP890667HU1
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Source Mammalian cell
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Product Details

Purity >85% (SDS-PAGE)
Target Names DUX4
Uniprot No. Q9UBX2
Alternative Names DUX4; DUX10Double homeobox protein 4; Double homeobox protein 10
Species Homo sapiens (Human)
Expression Region 327-424aa
Target Protein Sequence AGAAPPPQPAPPDASASARQGQMQGIPAPSQALQEPAPWSALPCGLLLDELLASPEFLQQAQPLLETEAPGELEASEEAASLEAPLSEEEYRALLEEL
Protein Length Partial
Tag Info The following tags are available.
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.
Form Lyophilized powder
Buffer before Lyophilization Tris/PBS-based buffer, 6% Trehalose, pH 8.0
Reconstitution 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.
Troubleshooting
and FAQs
Protein FAQs
Storage Condition Store at -20°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life 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.
Lead Time 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.
Notes 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.

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 Q&A
Q:

I am interested in your DUX4 Recombinant Protein; E. coli (0.5mg) (cat#: CSB-EP890667HU), and I would like to know if you can meet the following requirements:
1)At least 1mg/mL for the concentration; but if you could reach 2mg/mL that would be great?
2)Can you provide the protein with a small tag like His-tag for purification? If not then a tag that is at least cleavable is fine? Because they need the protein for functional assays and the tags might interfere with such kind assays.
3)Can you provide removal of myc-tag?

A:
Thanks for your inquiry.
1) Regarding your requirement for the concentration, we can try to provide the concentration of 2mg/mL, if not, at least to guarantee 1mg/mL.
2) We can preferentially choose a small purification tag such as N-terminal His-Tag, if it's not suitable, we will try to remove the tag.
If we succeed in removing the tag, we will charge for extra cost.
If we fail in removing the tag, we won’t charge for tag removal and provide the fusion protein, and remark this information in datasheet as follows
“Note: The laboratory determined that the Tag on your protein could not be removed with standard laboratory procedures. Your protein is being supplied with the Tag intact.”
Generally, the delivery time will be extended for 3 days. Please remark your requirements when placing the order.
3) We can guarantee to provide the protein without C-terminal tag, regarding the removal of N-terminal tag. we can try enzyme digestion, but we can't guarantee 100% successfully.

Target Data

Function Involved in transcriptional regulation. May regulate microRNA (miRNA) expression.
Gene References into Functions
  1. Sporadic DUX4 expression seen in FSHD myocytes is due to the incomplete repression by the PRC2 complex. PMID: 30122154
  2. Biallelic DUX4 expression lowers the threshold for disease presentation and is a modifier for disease severity in FSHD2. PMID: 29162933
  3. recurrent IGH-DUX4 or ERG-DUX4 fusions, ETV6-RUNX1-like gene-expression profile in B-cell precursor acute lymphoblastic leukaemia, is reported. PMID: 27265895
  4. The recent identification of aberrant activation of DUX4 transcription in Facioscapulohumeral muscular dystrophy. PMID: 29478599
  5. Case Report: t(10;19) CIC-DUX4 undifferentiated small round cell sarcoma of the abdominal wall. PMID: 28645808
  6. The DUX4 homeodomains mediate inhibition of myogenesis and are functionally exchangeable with the Pax7 homeodomain. PMID: 28935672
  7. selective loss of H3K9me3 from the DUX4 locus is associated with expression of DUX4 in late-phase squamous differentiation of human keratinocytes in vitro and in vivo. PMID: 26872601
  8. We discuss the involvement of this rearrangement in Facioscapulohumeral dystrophy (FSHD), since all mutations in SMCHD1 are not associated with D4Z4 hypomethylation and do not always segregate with the disease PMID: 28744936
  9. The study describes a model system for inducible DUX4 expression that enables reproducible and synchronized experiments and validates the fidelity and facioscapulohumeral dystrophy (FSHD) relevance of multiple distinct models of DUX4 expression. PMID: 28171552
  10. DUX4 and Dux may regulate some common pathways, and despite diverging from a common progenitor under different selective pressures for millions of years, the two genes maintain partial functional homology. PMID: 28173143
  11. We propose that DUX4 controls the cellular migration of mesenchymal stem cells through the CXCR4 receptor. PMID: 27556182
  12. These novel inhibitors of DUX4 transcriptional activity may thus act on pathways or cofactors needed by DUX4 for transcriptional activation in these cells. PMID: 27245141
  13. It has been proposed that the induction of DNA damage is a novel function of the DUX4 protein affecting myogenic differentiation of facioscapulohumeral dystrophy myoblasts. PMID: 27519269
  14. results underscore the complexity of the region immediately downstream of the D4Z4 and uncover a previously unknown function for the beta-satellite region in Dux4 cleavage and polyadenylation. PMID: 28540412
  15. Gene silencing of CIC-DUX4 as well as Ccnd2, Ret, and Bcl2 effectively inhibited CDS tumor growth in vitro PMID: 28404587
  16. Estrogens antagonize DUX4 transcriptional activity and its differentiation inhibitory function and support the protective role of these hormones toward facioscapulohumeral muscular dystrophy myoblast in in vitro differentiation. PMID: 28263188
  17. Findings show CIC-DUX4 sarcomas occur mostly in young adults within the somatic soft tissues, having a wide spectrum of morphology including round, epithelioid and spindle cells, and associated with an aggressive clinical course, with an inferior survival compared with Ewing sarcoma. Results support classification of CIC-rearranged tumors as an independent molecular and clinical subset of small blue round cell tumors. PMID: 28346326
  18. DUX4 activate genes associated with a cleavage-stage embryos in muscle cells. PMID: 28459454
  19. DUX4 role in activating cleavage-stage genes and MERVL/HERVL retrotransposons. PMID: 28459457
  20. DUX4 rearrangement and overexpression is associated with acute lymphoblastic leukemia. PMID: 27776115
  21. Targeted next-generation sequencing of CIC-DUX4 soft tissue sarcomas demonstrates low mutational burden and recurrent chromosome 1p loss. PMID: 27664537
  22. Transcriptomic analysis showed that DUX4 operates through both target gene activation and repression to orchestrate a transcriptome characteristic of a less-differentiated cell state. PMID: 27744317
  23. MYC, DUX4, and EIF4A3 might contribute to facioscapulohumeral dystrophy pathophysiology PMID: 28273136
  24. data shows that DUX4 can become an oncogenic driver as a result of somatic chromosomal rearrangements and that acute lymphoblastic leukemia in adolescents and young adults may be a clinical entity distinct from ALL at other ages PMID: 27019113
  25. We show that a DUX4 minigene, bearing only the homeodomains and C-terminus, is transcriptionally functional and cytotoxic, and that overexpression of a nuclear targeted C-terminus impairs the ability of WT DUX4 to interact with p300 and to regulate target genes. PMID: 26951377
  26. The aim of this study was to describe seven cases of CIC-DUX4 fusion-positive sarcomas, including the first reported example arising primarily in bone. Our series confirms that CIC-DUX4 fusion-positive sarcomas are aggressive tumours with an adverse prognosis PMID: 27079694
  27. CIC-DUX4 gene fusion is associated with Round cell sarcoma. PMID: 26800124
  28. Report DNA-binding sequence preferences of DUX4. PMID: 26823969
  29. Recent studies indicate that a combination of genetic and epigenetic factors that act on the D4Z4 repeat array determine the probability of DUX4 expression in skeletal muscle and disease penetrance and progression PMID: 26356006
  30. DUX4 mRNAs were induced during the differentiation of hMSCs into osteoblasts and that this process involved DUX4 and new longer protein forms. PMID: 26192274
  31. Endogenous DUX4 expression in FSHD myotubes is sufficient to cause cell death and disrupts RNA splicing and cell migration pathways. PMID: 26246499
  32. Interactions between DUX4 and DUX4c with cytoplasmic proteins play a major role during muscle differentiation. PMID: 26816005
  33. Loss of D4Z4 repression in facioscapulohumeral muscular dystrophy is observed as hypomethylation of the array accompanied by loss of repressive chromatin marks. [Review] PMID: 26113644
  34. Our results demonstrate that FRG1 is a direct DUX4 transcriptional target uncovering a novel regulatory circuit contributing to Facioscapulohumeral muscular dystrophy. PMID: 25326393
  35. This feedback loop illustrates an unexpected mode of autoregulatory behavior of a transcription factor, is consistent with 'bursts' of DUX4 expression in facioscapulohumeral muscular dystrophy muscle. PMID: 25564732
  36. These findings demonstrate that the expression of DUX4 accounts for the majority of the gene expression changes in facioscapulohumeral dystrophy skeletal muscle together with an immune cell infiltration. PMID: 24861551
  37. There is a special role of the 4q/10q D4Z4 chromatin and the DUX4 open reading frame in facioscapulohumeral muscular dystrophy. PMID: 24838473
  38. The distinct gene signature and immunoprofile of CIC-DUX4 sarcomas suggest a distinct pathogenesis from Ewing sarcoma. PMID: 24723486
  39. Results show that both DUX4-FL isoforms are expressed in facioscapulohumeral muscular dystrophy (FSHD) myotubes; DUX4-FL expression level is much lower in trapezius than in quadriceps myotubes, which is confirmed by the level of expression of DUX4 downstream genes PMID: 23966205

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Involvement in disease Facioscapulohumeral muscular dystrophy 1 (FSHD1)
Subcellular Location Nucleus
Protein Families Paired homeobox family
Tissue Specificity Does not seem to be expressed in normal muscle, but is detected in muscle of individuals with FSHD, and also in testis (at protein level) (PubMed:21060811). Does not seem to be expressed in normal muscle, but in muscle of individuals with FSHD, where it m
Database Links

HGNC: 50800

OMIM: 158900

KEGG: hsa:100288687

UniGene: Hs.728749

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