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- 详细信息
- 文献和实验
- 技术资料
- 免疫原:
Recombinant Human Cytochrome c oxidase subunit 6B1 protein (1-86AA)
- 亚型:
IgG
- 形态:
Liquid
- 保存条件:
Upon receipt, store at -20℃ or -80℃. Avoid repeated freeze.
- 克隆性:
Polyclonal
- 标记物:
Non-conjugated
- 适应物种:
Human
- 保质期:
6个月
- 抗原来源:
Homo sapiens (Human)
- 目录编号:
P14854
- 级别:
优
- 库存:
200
- 供应商:
武汉华美生物工程有限公司
- 宿主:
Rabbit
- 应用范围:
ELISA, IF; Recommended dilution: IF:1:50-1:200
- 浓度:
>95%,Protein G purified
- 靶点:
COX6B1
- 抗体英文名:
COX6B1 Antibody
- 抗体名:
Cytochrome c oxidase subunit Vib polypeptide 1 antibody
- 规格:
100μg/50μg/20μg
| 规格: | 100μg | 产品价格: | ¥1320.0 |
|---|---|---|---|
| 规格: | 50μg | 产品价格: | ¥880.0 |
| 规格: | 20μg | 产品价格: | ¥440.0 |
保存缓冲液
Preservative: 0.03% Proclin 300Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
功能
Defects in COX6B1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]; also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome.风险提示:丁香通仅作为第三方平台,为商家信息发布提供平台空间。用户咨询产品时请注意保护个人信息及财产安全,合理判断,谨慎选购商品,商家和用户对交易行为负责。对于医疗器械类产品,请先查证核实企业经营资质和医疗器械产品注册证情况。
文献和实验[资源] 所有的看家基因(housekeeping genes)列表+引物设计服务
, H+ transporting, lysosomal interacting protein 1 (ATP6IP1), mRNA 1089 NM_003526 Homo sapiens H2B histone family, member L (H2BFL), mRNA 2232 NM_004718 Homo sapiens cytochrome c oxidase subunit VIIa polypeptide 2 like (COX7A2L), nuclear gene
级联反应:细胞色素C/Apaf-1复合物激活caspase-9,后者再激活caspase-3和其它下游caspase。细胞色素C氧化酶亚单位Ⅳ(cytochrome c oxidase subunit Ⅳ:COX4)是定位在线粒体内膜上的膜蛋白,凋亡发生时,它保留在线粒体内,因而它是线粒体富集部分的一个非常有用的标志。 ApoAlertTMCell Fractionation Kit不用超离心,可从凋亡和非凋亡细胞中快速有效分离出高度富集的线粒体部分,再进一步通过Western杂交
线粒体荧光探针大全:TMRM,Mitotracker,JC-1
membrane-bound protein complexes, namely NADH�ubiquinol oxidoreductase (Complex I), succinate�ubiquinol oxidoreductase (Complex II), ubiquinol�cytochrome c oxidoreductase (Complex III), cytochrome c oxidase (Complex IV) and ATP synthase (Complex V
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