Recombinant Human Double Homeobox Protein 4 (DUX4) Protein (His)

Name: Recombinant Human Double Homeobox Protein 4 (DUX4) Protein (His)
Brand: Beta LifeScience
SKU: BLC-07825P
Availability: InStock

Greater than 85% as determined by SDS-PAGE.

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Product Overview

Description	Recombinant Human Double Homeobox Protein 4 (DUX4) Protein (His) is produced by our E.coli expression system. This is a protein fragment.
Purity	Greater than 85% as determined by SDS-PAGE.
Uniprotkb	Q9UBX2
Target Symbol	DUX4
Synonyms	Double homeobox protein 10
Species	Homo sapiens (Human)
Expression System	E.coli
Tag	N-6His
Target Protein Sequence	AGAAPPPQPAPPDASASARQGQMQGIPAPSQALQEPAPWSALPCGLLLDELLASPEFLQQAQPLLETEAPGELEASEEAASLEAPLSEEEYRALLEEL
Expression Range	327-424aa
Protein Length	partial
Mol. Weight	14.3 kDa
Research Area	Stem Cells
Form	Liquid or Lyophilized powder
Buffer	Liquid form: default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.
Reconstitution	Briefly centrifuged the vial prior to opening to bring the contents to the bottom. Reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL. It is recommended to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20°C/-80°C. The default final concentration of glycerol is 50%.
Storage	1. Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. 2. Avoid repeated freeze-thaw cycles. 3. Store working aliquots at 4°C for up to one week. 4. In general, protein in liquid form is stable for up to 6 months at -20°C/-80°C. Protein in lyophilized powder form is stable for up to 12 months at -20°C/-80°C.
Notes	Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.

Target Details

Target Function	Transcription factor that is selectively and transiently expressed in cleavage-stage embryos. Binds to double-stranded DNA elements with the consensus sequence 5'-TAATCTAATCA-3'. Binds to chromatin containing histone H3 acetylated at 'Lys-27' (H3K27ac) and promotes deacetylation of H3K27ac. In parallel, binds to chromatin that lacks histone H3 acetylation at 'Lys-27' (H3K27ac) and recruits EP300 and CREBBP to promote acetylation of histone H3 at 'Lys-27' at new sites. Involved in transcriptional regulation of numerous genes, primarily as transcriptional activator, but mediates also repression of a set of target genes. Promotes expression of ZSCAN4 and KDM4E, two proteins with essential roles during early embryogenesis. Heterologous expression in cultured embryonic stem cells mediates also transcription of HERVL retrotransposons and transcripts derived from ACRO1 and HSATII satellite repeats. May activate expression of PITX1. May regulate microRNA (miRNA) expression. Inappropriate expression can inhibit myogenesis and promote apoptosis.; Probably inactive as a transcriptional activator, due to the absence of the C-terminal region that is important for transcriptional activation. Can inhibit transcriptional activation mediated by isoform 1. Heterologous expression of isoform 2 has no deleterious effect on cell survival.
Subcellular Location	[Isoform 1]: Nucleus.; [Isoform 2]: Nucleus.
Protein Families	Paired homeobox family
Database References	HGNC: 50800 OMIM: 158900 KEGG: hsa:100288687 UniGene: Hs.728749
Associated Diseases	Facioscapulohumeral muscular dystrophy 1 (FSHD1)
Tissue Specificity	Isoform 1: 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). Isoform 1: Does not seem to be expressed in normal muscle, but in muscle of individuals with FSHD, where

Gene Functions References

Sporadic DUX4 expression seen in FSHD myocytes is due to the incomplete repression by the PRC2 complex. PMID: 30122154
Biallelic DUX4 expression lowers the threshold for disease presentation and is a modifier for disease severity in FSHD2. PMID: 29162933
recurrent IGH-DUX4 or ERG-DUX4 fusions, ETV6-RUNX1-like gene-expression profile in B-cell precursor acute lymphoblastic leukaemia, is reported. PMID: 27265895
The recent identification of aberrant activation of DUX4 transcription in Facioscapulohumeral muscular dystrophy. PMID: 29478599
Case Report: t(10;19) CIC-DUX4 undifferentiated small round cell sarcoma of the abdominal wall. PMID: 28645808
The DUX4 homeodomains mediate inhibition of myogenesis and are functionally exchangeable with the Pax7 homeodomain. PMID: 28935672
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
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
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
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
We propose that DUX4 controls the cellular migration of mesenchymal stem cells through the CXCR4 receptor. PMID: 27556182
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
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
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
Gene silencing of CIC-DUX4 as well as Ccnd2, Ret, and Bcl2 effectively inhibited CDS tumor growth in vitro PMID: 28404587
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
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
DUX4 activate genes associated with a cleavage-stage embryos in muscle cells. PMID: 28459454
DUX4 role in activating cleavage-stage genes and MERVL/HERVL retrotransposons. PMID: 28459457
DUX4 rearrangement and overexpression is associated with acute lymphoblastic leukemia. PMID: 27776115
Targeted next-generation sequencing of CIC-DUX4 soft tissue sarcomas demonstrates low mutational burden and recurrent chromosome 1p loss. PMID: 27664537
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
MYC, DUX4, and EIF4A3 might contribute to facioscapulohumeral dystrophy pathophysiology PMID: 28273136
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
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
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
CIC-DUX4 gene fusion is associated with Round cell sarcoma. PMID: 26800124
Report DNA-binding sequence preferences of DUX4. PMID: 26823969
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
DUX4 mRNAs were induced during the differentiation of hMSCs into osteoblasts and that this process involved DUX4 and new longer protein forms. PMID: 26192274
Endogenous DUX4 expression in FSHD myotubes is sufficient to cause cell death and disrupts RNA splicing and cell migration pathways. PMID: 26246499
Interactions between DUX4 and DUX4c with cytoplasmic proteins play a major role during muscle differentiation. PMID: 26816005
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
Our results demonstrate that FRG1 is a direct DUX4 transcriptional target uncovering a novel regulatory circuit contributing to Facioscapulohumeral muscular dystrophy. PMID: 25326393
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
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
There is a special role of the 4q/10q D4Z4 chromatin and the DUX4 open reading frame in facioscapulohumeral muscular dystrophy. PMID: 24838473
The distinct gene signature and immunoprofile of CIC-DUX4 sarcomas suggest a distinct pathogenesis from Ewing sarcoma. PMID: 24723486
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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Our protein products do not contain carrier protein or other additives (such as bovine serum albumin (BSA), human serum albumin (HSA) and sucrose, etc., and when lyophilized with the solution with the lowest salt content, they often cannot form A white grid structure, but a small amount of protein is deposited in the tube during the freeze-drying process, forming a thin or invisible transparent protein layer.

Reminder: Before opening the tube cap, we recommend that you quickly centrifuge for 20-30 seconds in a small centrifuge, so that the protein attached to the tube cap or the tube wall can be aggregated at the bottom of the tube. Our quality control procedures ensure that each tube contains the correct amount of protein, and although sometimes you can't see the protein powder, the amount of protein in the tube is still very precise.

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