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双同源框蛋白4抗体

供货周期： 7天

品牌： Abcam

型号： 0.1ml/100μg

货号：

报价： ¥1

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产品介绍

双同源框蛋白4抗体英文名称 Anti-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.

公司双同源框蛋白4抗体应用于医学免疫学、动物免疫学、分子生物学、生物化学、临床医学、检验医学、动物医学、药学、理工、农学、环境学等生命科学密切相关的专业。
浓度 1mg/1ml

规格 0.2ml/200μg

抗体来源 Rabbit

克隆类型 polyclonal

交叉反应 Human

产品类型一抗

研究领域细胞生物发育生物学信号转导干细胞

蛋白分子量 predicted molecular weight: 45kDa

性状 Lyophilized or Liquid

免疫原 KLH conjugated synthetic peptide derived from human DUX4

亚型 IgG

纯化方法 affinity purified by Protein A

储存液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500

（石蜡切片需做抗原修复）

not yet tested in other applications.

optimal dilutions/concentrations should be determined by the end user.

保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

双同源框蛋白4抗体产品介绍 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.

Database links : UniProtKB/Swiss-Prot: Q9UBX2.2

【存储要求】连续使用时4°C存储，保质期六个月；双同源框蛋白4抗体期存储时建议分装为10ul以上小包装-20°C存储，并避免反复冻融，保质期一年。

实验的用途：

1）WB：Western Blotting 免疫印迹

2）IH：Immunohistochemistry 免疫组化

3）IH(P)：Immunohistochemistry Parraffin sections 免疫组化(石蜡)

4）IH(F)：Immunohistochemistry Frozen sections 免疫组化(冰冻)

5）IC：Immunocytochemistry 免疫细胞化学