Rabbit Anti-Human BCS1L pAb
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产品编号
PA15893
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别名
BCS1L, BCS, BCS1, BJS, FLNMS, GRACILE, Hs.6719, MC3DN1, PTD, h-BCS, h-BCS1
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规格
- 50ul
- 100ul
Alias | BCS1L, BCS, BCS1, BJS, FLNMS, GRACILE, Hs.6719, MC3DN1, PTD, h-BCS, h-BCS1 |
Catalog Number | PA15893 |
Molecular Name | BCS1L |
Host | Rabbit |
Size | 50ul, 100ul |
Clonity | Polyclonal |
Purification | Affinity Purification |
Formulation | Supply in PBS with 0.02% sodium azide, 50% glycerol, pH 7.4. |
Immunogen | Recombinant fusion protein containing a sequence corresponding to a.a. 33-240 of human BCS1L (NP_004319.1). |
Application | WB, IHC, IF |
Storage Condition | Store the product under sterile conditions after opening at -20℃. Avoid freeze-thaw cycles. |
Shipping Condition | Shipped on ice packs. |
Product Declaration | 本产品仅用于研究用途(RUO)。不能用于诊断或直接用于人体实验。 |
Product Full Name | Rabbit Anti-Human BCS1L Polyclonal Antibody |
BCS1L(线粒体复合体III组装因子)是线粒体呼吸链复合体III(细胞色素bc1复合体)的关键组装因子,负责将细胞色素c1和Rieske铁硫蛋白整合到复合体中。其功能异常会导致氧化磷酸化障碍,影响ATP生成。
可以,推荐使用BCS1L突变患者的成纤维细胞或CRISPR敲除细胞模型,通过Blue Native PAGE检测复合体III组装缺陷,并结合耗氧率分析(Seahorse)评估线粒体功能。
经测试无交叉反应,特异性针对BCS1L,不识别BCS1A或其他线粒体组装因子。