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产品信息
MA5-16147
应用
建议稀释比
已发表文章
产品规格
种属反应
Human
已发表种属
Human
宿主/亚型
Mouse
/ IgG1
分类
Monoclonal
类型
Antibody
克隆号
P4H2
抗原
Synthetic peptide corresponding to the C-terminal of human DUX4.
偶联物
形式
Liquid
浓度
1 mg/mL
纯化类型
Protein G
保存液
PBS
内含物
0.02% sodium azide
保存条件
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
RRID
AB_11152574
靶标信息
DUX4 (also known as Double homeobox 4) is the leading candidate causative gene for facioscapulohumeral dystrophy (FSHD), a degenerative skeletal muscle disease and one of the most common muscular dystrophies. FSHD is caused by the deletion of a subset of D4Z4 macrosatellite repeats on chromosome 4. Each repeat contains a retrogene encoding the double-homeobox factor DUX4. DUX4 expression is epigenetically suppressed in differentiated tissues and the residual DUX4 transcripts are spliced to remove the carboxyterminal domain that has been associated with cell toxicity. In FSHD individuals, the expression of the full-length DUX4 transcript is not completely suppressed in skeletal muscle, and possibly other differentiated tissues, and results in a small percentage of cells expressing relatively abundant amounts of the full-length DUX4 mRNA and protein.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
生物信息学
蛋白别名: double homeobox 4-like; Double homeobox protein 10; Double homeobox protein 4; double homeobox protein 4/10
基因别名: DUX10; DUX4; DUX4L
UniProt ID: (Human) Q9UBX2
Entrez Gene ID: (Human) 100288687
