NDUFA4 Antibody
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货号:CSB-PA955890
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规格:¥2024
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图片:
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其他:
产品详情
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产品名称:Rabbit anti-Homo sapiens (Human) NDUFA4 Polyclonal antibody
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Uniprot No.:O00483
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基因名:
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宿主:Rabbit
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反应种属:Human
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免疫原:Synthesized peptide derived from internal of Human NDUFA4.
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免疫原种属:Homo sapiens (Human)
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克隆类型:Polyclonal
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纯化方式:The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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浓度:It differs from different batches. Please contact us to confirm it.
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产品提供形式:Liquid
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应用范围:ELISA,WB
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推荐稀释比:
Application Recommended Dilution WB 1:500-1:3000 -
Protocols:
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储存条件:Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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货期:Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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靶点详情
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功能:Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Electrons originating from reduced cytochrome c in the intermembrane space (IMS) are transferred via the dinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1 to the active site in subunit 1, a binuclear center (BNC) formed by heme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2 water molecules unsing 4 electrons from cytochrome c in the IMS and 4 protons from the mitochondrial matrix. NDUFA4 is required for complex IV maintenance.
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基因功能参考文献:
- Findings support reassignment of the NDUFA4 protein to complex IV and suggest that patients with unexplained COX deficiency should be screened for NDUFA4 mutations. PMID: 23746447
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相关疾病:Leigh syndrome (LS)
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亚细胞定位:Mitochondrion inner membrane; Single-pass membrane protein.
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蛋白家族:Complex IV NDUFA4 subunit family
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数据库链接:
HGNC: 7687
OMIM: 256000
KEGG: hsa:4697
STRING: 9606.ENSP00000339720
UniGene: Hs.50098
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