BBS4 抗体 (AA 431-519) (AbBy Fluor® 488)
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- 抗原 See all BBS4 抗体
- BBS4 (Bardet-Biedl Syndrome 4 (BBS4))
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抗原表位
- AA 431-519
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适用
- 小鼠
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宿主
- 兔
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克隆类型
- 多克隆
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标记
- This BBS4 antibody is conjugated to AbBy Fluor® 488
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应用范围
- Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- 交叉反应
- 小鼠
- 预测反应
- Human,Rat,Dog,Cow,Sheep,Pig,Horse
- 纯化方法
- Purified by Protein A.
- 免疫原
- KLH conjugated synthetic peptide derived from human BBS4
- 亚型
- IgG
- Top Product
- Discover our top product BBS4 Primary Antibody
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- 应用备注
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 - 限制
- 仅限研究用
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- 状态
- Liquid
- 浓度
- 1 μg/μL
- 缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
- 储存液
- ProClin
- 注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- 储存条件
- -20 °C
- 储存方法
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
- 有效期
- 12 months
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- 抗原
- BBS4 (Bardet-Biedl Syndrome 4 (BBS4))
- 别名
- BBS4 (BBS4 产品)
- 别名
- CG13232 antibody, Dmel\\CG13232 antibody, zgc:152964 antibody, AW537059 antibody, AW742241 antibody, D9Ertd464e antibody, Bardet-Biedl syndrome 4 antibody, bardet-biedl syndrome 4 antibody, Bardet-Biedl syndrome 4 (human) antibody, BBS4 antibody, bbs4 antibody, Bbs4 antibody
- 背景
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Synonyms: Bardet Biedl syndrome 4 protein, Bardet-Biedl syndrome 4 protein, Bbs4, BBS4_HUMAN.
Background: Bardet-Biedl syndrome (BBS) is a pleiotropic genetic disorder characterized by obesity, photoreceptor degeneration, polydactyly, hypogenitalism, renal abnormalities, and developmental delay. Other associated clinical findings in BBS patients include diabetes, hypertension, and congenital heart defects. BBS is a heterogeneous disorder, BBS genes map to eight genetic loci and encode eight proteins, BBS1-BBS8. Five BBS genes encode basal body or cilia proteins, suggesting that BBS is a ciliary dysfunction disorder. BBS4 is expressed in the olfactory epithelium and localizes to the centriolar satellites of centrosomes and basal bodies of primary cilia. BBS4 regulates the p150 subunit of the dynein transport machinery (DCTN1) to attract pericentriolar material-1 protein (PCM1) and its associated components to the satellites. Loss of BBS4 is correlated with obesity caused by abnormal lipid profiles, liver dysfunction, elevated insulin, and abnormal leptin levels.
- 途径
- Hedgehog Signaling, Tube Formation, Maintenance of Protein Location
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