NDUFS4 Antibody
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中文名稱：NDUFS4兔多克隆抗體
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貨號：CSB-PA048629
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規格：￥1100
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圖片：
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Gel: 10%SDS-PAGE, Lysate: 40 μg, Lane 1-2: Mouse heart tissue, Mouse kidney tissue, Primary antibody: CSB-PA048629(NDUFS4 Antibody) at dilution 1/200, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 20 seconds
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其他：
產品詳情
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Uniprot No.：
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基因名：
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別名：AQDQ antibody; CI 18 antibody; CI 18 kDa antibody; CI AQDQ antibody; CI-18 kDa antibody; CI-AQDQ antibody; Complex I 18 kDa antibody; Complex I AQDQ antibody; Complex I-18 kDa antibody; Complex I-AQDQ antibody; mitochondrial antibody; mitochondrial respiratory chain complex I (18 KD subunit) antibody; NADH coenzyme Q reductase antibody; NADH dehydrogenase (ubiquinone) Fe S protein 4 18kDa antibody; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4 antibody; NADH dehydrogenase antibody; NADH ubiquinone oxidoreductase 18 kDa subunit antibody; NADH-ubiquinone oxidoreductase 18 kDa subunit antibody; NDUFS4 antibody; NDUS4_HUMAN antibody
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宿主：Rabbit
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反應種屬：Human,Mouse,Rat
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免疫原：Fusion protein of Human NDUFS4
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免疫原種屬：Homo sapiens (Human)
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標記方式：Non-conjugated
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抗體亞型：IgG
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純化方式：Antigen affinity purification
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
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產品提供形式：Liquid
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應用范圍：ELISA,WB
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推薦稀釋比：
Application Recommended Dilution ELISA 1:2000-1:5000 WB 1:500-1:2000 -
Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：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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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
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基因功能參考文獻：
- The clinical presentations of five individuals of Hutterite descent with Leigh disease are described herein. An identity-by-descent mapping and candidate gene approach was used to identify a novel homozygous c.393dupA frameshift mutation in the NADH dehydrogenase (ubiquinone) Fe-S protein 4 (NDUFS4) gene. PMID: 27671926
- The authors concluded that NDUFS4-related Leigh syndrome is invariably linked to an early onset severe phenotype that results in early death. PMID: 27079373
- The c.462delA deletion led to a complete lack of NDUFS4 peptide in isolated mitochondria, and this deficiency caused an inefficient mitochondrial complex I assembly and Leigh syndrome symptoms. PMID: 24020637
- Mutations in the NDUFS4 gene and its subunits are associated with the mitochondrial complex I deficiency. (Review) PMID: 23378164
- Elevated expression of CO I and ND4 were associated with gastric tumorigenesis and tumor dedifferentiation PMID: 22407105
- Studies indicate that that the functional capacity of complex I depends on phosphorylation and import of subunit NDUFS4 protein. PMID: 21945319
- In fibroblast cultures, protein kinase A-mediated phosphorylation of the NDUFS4 subunit of complex I rescues the activity of the oxidatively damaged complex. PMID: 22198267
- case Report: A novel mutation in NDUFS4 causes Leigh syndrome in an Ashkenazi Jewish family. PMID: 19107570
- REVIEW: Phosphorylation of the NDUFS4 protein, overall respiratory activity, and mutations sassociated with deficiency of complex I. PMID: 11860175
- In patients with complex I deficiency, the increased whole-body oxygen consumption rate at rest reflects increased electron transport through the respiratory chain, driven by a decreased phosphorylation potential. PMID: 11940698
- observations show the essential role of the 18-kDa subunit of respiratory complex I (NDUFS4) gene in the structure and function of complex I and give insight into pathogenic mechanism of NDUFS4 gene mutations in a severe defect of complex I PMID: 12944388
- A nonsense mutation leading to the abrogation of mRNA decay was found in NDUFS4 gene of a Leigh syndrome patient. PMID: 15975579
- NDUSF4 is required for the assembly and stabilization of a portion of complex I that contains a number of subunits. PMID: 17438127
- impact of PKA mediated phosphorylation on the mitochondrial import of in vitro and in vivo synthesized NDUFS4 protein PMID: 18291624
- regulation of alternative transcripts of the NDUFS4 gene of complex I of the respiratory chain PMID: 18555024
- NDUFS4 presents a hotspot of mutations in the genetic apparatus of oxidative phosphorylation and the correct assembly of the subunit it encodes is essential for completion of the assembly of complex I. PMID: 19364667
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相關疾病：Mitochondrial complex I deficiency (MT-C1D); Leigh syndrome (LS)
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亞細胞定位：Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
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蛋白家族：Complex I NDUFS4 subunit family
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數據庫鏈接：
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