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Rabbit Anti-DUX4  antibody (bs-12369R)
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-12369R
英文名称 DUX4
中文名称 双同源框蛋白4抗体
别    名 Double homeobox protein 10; Double homeobox protein 4; Double homeobox protein 4/10; DUX10; DUX4_HUMAN.  
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.  
研究领域 细胞生物  发育生物学  信号转导  干细胞  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human)
产品应用 WB=1:500-2000, IHC-P=1:100-500, IHC-F=1:100-500, ICC=1:100-500, IF=1:100-500, ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 45kDa
细胞定位 细胞核 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DUX4: 53-120/424 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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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