產(chǎn)品編號 | bs-12369R |
英文名稱 | DUX4 Rabbit pAb |
中文名稱 | 雙同源框蛋白4抗體 |
別 名 | Double homeobox protein 10; Double homeobox protein 4; Double homeobox protein 4/10; DUX10; DUX4_HUMAN. |
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Specific References (1) | bs-12369R has been referenced in 1 publications.
[IF=10.392] Hao Zhang. et al. DNA crosslinking and recombination-activating genes 1/2 (RAG1/2) are required for oncogenic splicing in acute lymphoblastic leukemia. 2021 Oct 26 other ; Human.
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研究領(lǐng)域 | 細(xì)胞生物 發(fā)育生物學(xué) 信號轉(zhuǎn)導(dǎo) 干細(xì)胞 |
抗體來源 | Rabbit |
克隆類型 | Polyclonal |
交叉反應(yīng) | (predicted: Human) |
產(chǎn)品應(yīng)用 | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
理論分子量 | 45 kDa |
檢測分子量 | |
細(xì)胞定位 | 細(xì)胞核 |
性 狀 | Liquid |
濃 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human DUX4: 53-120/424 |
亞 型 | IgG |
純化方法 | affinity purified by Protein A |
緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
PubMed | PubMed |
產(chǎn)品介紹 |
DUX4 is a homeodomain protein with a similar protein sequence to Pax3 and Pax7. Defects in DUX4 may be the cause of facioscapulohumeral muscular dystrophy (FSHD). FSHD is characterized by weakness of the muscles of the face, upper-arm and shoulder girdle. Severity is highly variable. Weakness is slowly progressive and about 20% of affected individuals eventually require a wheelchair. Approximately 70-90% of individuals have inherited the disease-causing deletion from a parent, and approximately 10-30% of affected individuals have FSHD as the result of a de novo deletion. Offsprings of an affected individual have a 50% chance of inheriting the deletion. Function: May be involved in transcriptional regulation. Subunit: May exist as a monomer or a dimer. Subcellular Location: Nucleus. Note=Actively transported through the nuclear pore complex (NPC). Tissue Specificity: Does not seem to be expressed in normal muscle, but in muscle of individuals with FSHD, where it may be toxic to cells. DISEASE: Defects in DUX4 may be the cause of facioscapulohumeral muscular dystrophy (FSHD) [MIM:158900]. FSHD is characterized by weakness of the muscles of the face, upper-arm and shoulder girdle. Severity is highly variable. Weakness is slowly progressive and about 20% of affected individuals eventually require a wheelchair. Approximately 70-90% of individuals have inherited the disease-causing deletion from a parent, and approximately 10-30% of affected individuals have FSHD as the result of a de novo deletion. Offsprings of an affected individual have a 50% chance of inheriting the deletion. Similarity: Belongs to the paired homeobox family. Contains 2 homeobox DNA-binding domains. SWISS: Q9UBX2 Gene ID: 100288687 Database links: Entrez Gene: 100288687 Human Entrez Gene: 664783 Mouse Omim: 606009 Human SwissProt: Q9UBX2 Human Unigene: 553518 Human |
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