NDUFS4 Antibody, FITC conjugated

說明書

中文名稱：

NDUFS4兔多克隆抗體, FITC偶聯(lián)
貨號：

CSB-PA015663LC01HU
規(guī)格：

￥880
其他：

產(chǎn)品詳情
產(chǎn)品評價
相關產(chǎn)品
靶點詳情

產(chǎn)品詳情

產(chǎn)品名稱：

Rabbit anti-Homo sapiens (Human) NDUFS4 Polyclonal antibody
Uniprot No.：

O43181
基因名：

NDUFS4
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
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial protein (43-161AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

FITC
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產(chǎn)品提供形式：

Liquid
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評價

靶點詳情

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.
基因功能參考文獻：
1. 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
2. The authors concluded that NDUFS4-related Leigh syndrome is invariably linked to an early onset severe phenotype that results in early death. PMID: 27079373
3. 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
4. Mutations in the NDUFS4 gene and its subunits are associated with the mitochondrial complex I deficiency. (Review) PMID: 23378164
5. Elevated expression of CO I and ND4 were associated with gastric tumorigenesis and tumor dedifferentiation PMID: 22407105
6. Studies indicate that that the functional capacity of complex I depends on phosphorylation and import of subunit NDUFS4 protein. PMID: 21945319
7. 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
8. case Report: A novel mutation in NDUFS4 causes Leigh syndrome in an Ashkenazi Jewish family. PMID: 19107570
9. REVIEW: Phosphorylation of the NDUFS4 protein, overall respiratory activity, and mutations sassociated with deficiency of complex I. PMID: 11860175
10. 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
11. 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
12. A nonsense mutation leading to the abrogation of mRNA decay was found in NDUFS4 gene of a Leigh syndrome patient. PMID: 15975579
13. NDUSF4 is required for the assembly and stabilization of a portion of complex I that contains a number of subunits. PMID: 17438127
14. impact of PKA mediated phosphorylation on the mitochondrial import of in vitro and in vivo synthesized NDUFS4 protein PMID: 18291624
15. regulation of alternative transcripts of the NDUFS4 gene of complex I of the respiratory chain PMID: 18555024
16. 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)
亞細胞定位：

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
蛋白家族：

Complex I NDUFS4 subunit family
數(shù)據(jù)庫鏈接：

HGNC: 7711

OMIM: 252010

KEGG: hsa:4724

STRING: 9606.ENSP00000296684

UniGene: Hs.528222

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